RESUMEN
INTRODUCTION: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. METHODS: The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. RESULTS: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. CONCLUSIONS: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.
Asunto(s)
Procedimiento de Fontan , Puente Cardíaco Derecho , Cardiopatías Congénitas , Atresia Tricúspide , Tronco Arterial Persistente , Corazón Univentricular , Embarazo , Masculino , Humanos , Femenino , Lactante , Corazón Univentricular/cirugía , Cardiopatías Congénitas/cirugía , Puente Cardíaco Derecho/métodos , Arteria Pulmonar/cirugía , Arteria Pulmonar/anomalías , Atresia Tricúspide/cirugía , Tronco Arterial Persistente/cirugía , Estudios Retrospectivos , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/anomalías , Resultado del TratamientoRESUMEN
OBJECTIVES: To assess the short- and long-term outcomes of balloon pulmonary valvuloplasty (BPV) in children with Noonan syndrome (NS). BACKGROUND: Pulmonary stenosis (PS) is the most common congenital heart lesion in NS. BPV is the accepted first line treatment in PS. However, BPV in NS patients has been reported to be less effective, without specific factors for the need for reintervention being identified. METHODS: Retrospective case-note review of all patients with NS who underwent BPV between 1985 and 2020. Patients were divided into 2 groups: those with supravalvular pulmonary stenosis (SPS) in addition to valvar PS, and those with isolated valvar PS. RESULTS: A cohort of 54 patients with NS underwent BPV at a median of 275 (interquartile range [IQR]: 108-575) days of age. SPS was present in 32 (59%) patients whereas 22 had (41) isolated PS. The preprocedural invasive gradient was 47 (IQR: 35-69) mmHg, and 44 (IQR: 35-48) mmHg in those with SPS and those without respectively (p = 0.88). Reintervention was required in 22 patients (41%): 17 (77%) with SPS and 5 (23%) without (p = 0.017). Fourteen patients (11 with SPS) required surgical reintervention and 8 (6 with SPS) required further BPV. There was no significant difference in the age at initial BPV, pre- and postprocedural gradients and interval until reintervention between groups. CONCLUSION: This is the largest reported cohort of patients with NS undergoing BPV. Although BPV is often successful, the reintervention rates are high. SPS was a risk factor for reintervention.
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Síndrome de Noonan , Estenosis de la Válvula Pulmonar , Válvula Pulmonar , Niño , Humanos , Síndrome de Noonan/complicaciones , Síndrome de Noonan/diagnóstico , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Estenosis de la Válvula Pulmonar/diagnóstico por imagen , Estenosis de la Válvula Pulmonar/terapia , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
In patients with transposition, a right aortic arch has rarely been reported among the associated malformations. When present, the arch has been obstructed because of the presence of aortic coarctation and/or arch hypoplasia. A bicuspid aortic valve is also very rare in the presence of transposition. We report here the unusual association of transposition, an unobstructed right aortic arch, and a bicuspid aortic valve in a neonate who underwent a successful arterial switch operation.
Asunto(s)
Aorta Torácica/cirugía , Coartación Aórtica/cirugía , Operación de Switch Arterial , Enfermedad de la Válvula Aórtica Bicúspide/cirugía , Transposición de los Grandes Vasos/cirugía , Anomalías Múltiples/cirugía , Aorta Torácica/diagnóstico por imagen , Coartación Aórtica/diagnóstico por imagen , Enfermedad de la Válvula Aórtica Bicúspide/diagnóstico por imagen , Humanos , Recién Nacido , Complicaciones Posoperatorias , Transposición de los Grandes Vasos/diagnóstico por imagenRESUMEN
Right aortic arch (RAA) and retro-aortic innominate vein are rare vascular anomalies. Diagnosis of these anatomical variations can be achieved using fetal echocardiography, post-natal echocardiography, and computed tomography scan. RAA can form a vascular ring when associated with other vascular anomalies which may compress the trachea and/or esophagus. On the other hand, the existence of retro-aortic innominate vein can influence the clinical decision-making and surgical strategy. We report a rare occurrence of both RAA and retro-aortic innominate vein in a 3 months old girl with a prenatal diagnosis of tetralogy of Fallot and include details of her presentation and successful management.
RESUMEN
BACKGROUND: Infants born with cardiac abnormalities causing dependence on the arterial duct for pulmonary blood flow are often palliated with a shunt usually between the subclavian artery and either pulmonary artery. A so-called modified Blalock-Taussig shunt allows progress through early life to an age and weight at which repair or further more stable palliation can be safely achieved. Modified Blalock-Taussig shunts continue to present concern for postprocedural instability and early mortality such that other alternatives continue to be explored. Duct stenting (DS) is emerging as one such alternative with potential for greater early stability and improved survival. METHODS: The purpose of this study was to compare postprocedural outcomes and survival to next-stage palliative or reparative surgery between patients undergoing a modified Blalock-Taussig shunt or a DS in infants with duct-dependent pulmonary blood flow. All patients undergoing cardiac surgery and congenital interventions in the United Kingdom are prospectively recruited to an externally validated national outcome audit. From this audit, participating UK centers identified infants <30 days of age undergoing either a Blalock-Taussig shunt or a DS for cardiac conditions with duct-dependent pulmonary blood flow between January 2012 and December 31, 2015. One hundred seventy-one patients underwent a modified Blalock-Taussig shunt, and in 83 patients, DS was attempted. Primary and secondary outcomes of survival and need for extracorporeal support were analyzed with multivariable logistic regression. Longer-term mortality before repair and reintervention were analyzed with Cox proportional hazards regression. All multivariable analyses accommodated a propensity score to balance patient characteristics between the groups. RESULTS: There was an early (to discharge) survival advantage for infants before next-stage surgery in the DS group (odds ratio, 4.24; 95% confidence interval, 1.37-13.14; P=0.012). There was also a difference in the need for postprocedural extracorporeal support in favor of the DS group (odds ratio, 0.22; 95% confidence interval, 0.05-1.05; P=0.058). Longer-term survival outcomes showed a reduced risk of death before repair in the DS group (hazard ratio, 0.25; 95% confidence interval, 0.07-0.85; P=0.026) but a slightly increased risk of reintervention (hazard ratio, 1.50; 95% confidence interval, 0.85-2.64; P=0.165). CONCLUSIONS: DS is emerging as a preferred alternative to a surgical shunt for neonatal palliation with evidence for greater postprocedural stability and improved patient survival to destination surgical treatment.
Asunto(s)
Procedimiento de Blalock-Taussing , Cateterismo Cardíaco/instrumentación , Conducto Arterioso Permeable/cirugía , Cuidados Paliativos/métodos , Circulación Pulmonar , Stents , Aortografía , Procedimiento de Blalock-Taussing/efectos adversos , Procedimiento de Blalock-Taussing/mortalidad , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/mortalidad , Conducto Arterioso Permeable/diagnóstico por imagen , Conducto Arterioso Permeable/mortalidad , Conducto Arterioso Permeable/fisiopatología , Femenino , Mortalidad Hospitalaria , Humanos , Lactante , Mortalidad Infantil , Recién Nacido , Masculino , Auditoría Médica , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Reino UnidoRESUMEN
OBJECTIVES: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg. BACKGROUND: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited. METHODS: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications. RESULTS: A total of 408 patients underwent attempted transcatheter PDA closure between January 2004 and December 2014. The mean weight at catheterization was 4.9 ± 1.0 kg and mean age was 5.7 ± 3.0 months. Successful device implantation was achieved in 374 (92%) patients without major complication and of these, complete occlusion was achieved in 356 (95%) patients at last available follow-up. Device embolization occurred in 20 cases (5%). The incidence of device related obstruction to the left pulmonary artery or aorta and access related peripheral vascular injury were low. There were no deaths related to the procedure. CONCLUSIONS: Transcatheter closure of PDA can be accomplished in selected infants weighing <6 kg despite the manufacturer's recommended weight limit of 6 kg for most ductal occluders. The embolization rate is higher than previously reported in larger patients. Retrievability of the occluder and duct morphology needs careful consideration before deciding whether surgical ligation or transcatheter therapy is the better treatment option.