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1.
Radiol Cardiothorac Imaging ; 6(3): e230403, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38900025

RESUMO

Generalized arterial calcification of infancy (GACI) is a rare genetic condition with varied clinical presentation. Consequently, diagnosis is frequently delayed or missed. GACI has a poor prognosis, with more than half of patients dying before the age of 6 months. Early diagnosis and treatment with bisphosphonates have been shown to improve survival in these patients. This is a case report of a newborn with respiratory distress who was initially diagnosed with coarctation of the aorta at echocardiography. Further imaging with CT revealed the aortic narrowing to be associated with GACI. Keywords: Genetic Defects, Congenital, Vascular, Calcification/Calculi, Aorta, Pulmonary Arteries, CT Angiography, Echocardiography, Pediatrics © RSNA, 2024.


Assuntos
Coartação Aórtica , Ecocardiografia , Calcificação Vascular , Humanos , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/diagnóstico , Recém-Nascido , Calcificação Vascular/diagnóstico por imagem , Calcificação Vascular/diagnóstico , Diagnóstico Diferencial , Masculino , Angiografia por Tomografia Computadorizada , Feminino
2.
Artigo em Inglês | MEDLINE | ID: mdl-38752879

RESUMO

We describe a surgical technique for a half-turned truncal switch operation in a 5-year-old child with dextro-transposition of the great arteries (D-TGA), a ventricular septal defect, a left ventricular outflow tract obstruction and a complex coronary pattern. The benefit of the half-turned truncal switch is the creation of haemodynamically superior biventricular outflow tracts and the maximal use of an autologous pulmonary valve in the right ventricular outflow tract, thereby avoiding the right ventricular-pulmonary artery conduit.


Assuntos
Transposição das Grandes Artérias , Comunicação Interventricular , Transposição dos Grandes Vasos , Obstrução do Fluxo Ventricular Externo , Humanos , Transposição dos Grandes Vasos/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Comunicação Interventricular/cirurgia , Pré-Escolar , Transposição das Grandes Artérias/métodos , Masculino , Anormalidades Múltiplas/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Obstrução da Via de Saída Ventricular Esquerda
3.
World J Pediatr Congenit Heart Surg ; : 21501351231207093, 2023 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-37976423

RESUMO

Double outlet of both ventricles is an anomaly wherein both ventricles equally share the arterial trunks. A majority of the literature describes a variant of this disease with a muscular outlet septum which is perpendicular to the plane of the ventricular septum although a variant with a fibrous muscular septum can also be present. The condition may be associated with obstruction of either outflow tract, which may complicate repair. We report a child with double outlet of both ventricles and a fibrous outlet septum with unobstructed outflows. The child underwent a successful surgical repair using a two-patch technique.

4.
BMJ Glob Health ; 8(5)2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-37142298

RESUMO

The global burden of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs. Effective and safe PCHD care is inaccessible to many, and there is no consensus on the best approaches to provide meaningful access in resource-limited settings, where it is often needed the most. Considering the high inequity in access to care for CHD and RHD, we aimed to create an actionable framework for health practitioners, policy makers and patients that supports treatment and prevention. It was formulated based on rigorous evaluation of available guidelines and standards of care and builds on a consensus process about the competencies needed at each step of the care continuum. We recommend a tier-based framework for PCHD care integrated within existing health systems. Each level of care is expected to meet minimum benchmarks and ensure high-quality and family centred care. We propose that cardiac surgery capabilities should only be developed at the more advanced levels on hospitals that have an established foundation of cardiology and cardiac surgery services, including screening, diagnostics, inpatient and outpatient care, postoperative care and cardiac catheterisation. This approach requires a quality control system and close collaboration between the different levels of care to facilitate the journey and care of every child with heart disease. This effort was designed to guide readers and leaders in taking action, strengthening capacity, evaluating impact, advancing policy and engaging in partnerships to guide facilities providing PCHD care in LMICs.


Assuntos
Países em Desenvolvimento , Cardiopatias Congênitas , Humanos , Criança , Saúde Pública , Cardiopatias Congênitas/cirurgia , Sistema de Registros , Continuidade da Assistência ao Paciente
5.
Ann Pediatr Cardiol ; 16(6): 399-406, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38817266

RESUMO

Objective: To study the applicability of on-table extubation (OTE) protocol following congenital cardiac surgery in a low-resource setting and its impact on the length of intensive care unit (ICU) stay, hospital stay, hospitalization cost, parental anxiety, and nurse anxiety. Materials and Methods: In this prospective, nonrandomized, observational single-center study, we included all children above 1 year of age undergoing congenital cardiac surgery. We evaluated them for the feasibility of OTE using a prespecified protocol following separation from cardiopulmonary bypass. The data were prospectively collected on 60 children more than 1 year of age, belonging to the Risk Adjustment for Congenital Heart Surgery 1, 2, 3, and 4 groups and divided into two groups: those who underwent successful OTE and those who were ventilated for any duration postoperatively (30 children in each group). Duration of hospital stay, ICU stay, and total hospital cost were collected. Anxiety levels of the primary caregiver (nurse) in the ICU and the mother were assessed immediately after the arrival of the child in the ICU using the State Trait Anxiety Inventory (STAI). Results: Children who were extubated immediately following congenital cardiac surgery had significantly shorter ICU stay (median 20 [19, 22] h vs. 22 [20, 43] h [P < 0.05]). Patients extubated on table had a significant reduction in hospital cost {median Rs. 161,000 (138,330; 211,900), approximately USD 1970 (P < 0.05)} when compared to children who were ventilated postoperatively {median Rs. 201,422 (151,211; 211,900) , approximately USD 2464}. The anxiety level in mothers was significantly less when their child was extubated in the operating room (STAI 36.5 ± 5.4 vs. 47.4 ± 7.4, P < 0.001). However, for the same subset of patients, anxiety level was significantly higher in the ICU nurse (STAI 46.0 ± 5.6 vs. 37.8 ± 4.1, P < 0.05). Conclusion: OTE following congenital cardiac surgery is associated with a shorter duration of ICU stay and hospital stay. It also reduces the total hospital cost and the anxiety level in mothers of children undergoing congenital heart surgery. However, the primary bedside caregiver during the child's ICU stay had increased anxiety managing patients with OTE.

6.
Indian J Thorac Cardiovasc Surg ; 38(2): 173-176, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35221555

RESUMO

The association of pulmonary venous anomaly and a complex complete transposition of great arteries (d-TGA) is a rarity. Managing a combination of anomalies in a single setting is a surgical challenge. We report a very rare association of d-TGA with ventricular septal defect (VSD) and partial anomalous pulmonary venous connection (PAPVC) of the right pulmonary veins to the right atrium with an intact interatrial septum in a month-old infant. The child underwent a single-stage complete repair. Constant vigilance in the immediate postoperative period and early revision is mandatory for a good outcome.

7.
World J Pediatr Congenit Heart Surg ; 12(3): 406-410, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33942692

RESUMO

The modified Ozaki technique within a conduit is reproducible and is an alternative to the use of homografts and commercially available conduits which may have special appeal in a low resource setting.


Assuntos
Bioprótese , Artéria Pulmonar , Aorta/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Estudos Retrospectivos
8.
Ann Card Anaesth ; 24(2): 217-223, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33884979

RESUMO

Background: Prophylactic milrinone is commonly used to prevent Low Cardiac Output Syndrome (LCOS) after pediatric cardiac surgery. This study compares the use of levosimendan with milrinone when used as the primary inotrope following pediatric cardiac surgery. Subjects and Methods: Forty infants undergoing corrective surgery for congenital heart disease were recruited during the study and randomized into two groups (group L and group M). During rewarming, a loading dose of levosimendan or milrinone was administered followed by a 24-hour infusion of the chosen inotrope. Echocardiographic variables were measured postoperatively. Statistical analysis was done with SPSS-20 computer package. Association between the variables was found by independent t test. P < 0.05 was considered statistically significant. Results: Mean age and weight of the patient in Group L was 8.55 ± 5.83 months and 6.05 ± 2.09 kgs, while that in group M was 6.85 ± 3.57 months and 5.26 ± 2.11 kgs. 4 patients (20%) treated with levosimendan had LCOS in comparison with 6 (30%) patients in those treated with milrinone. Echocardiographic parameters in both groups L and M were comparable (cardiac index 3.47 ± 0.76 vs 3.72 ± 1.05 L/min/m2, EF 66.10 ± 7.82% vs 59.34 ± 10.74%, stroke volume index 25.4 ± 6.3 vs 27.74 ± 10.35 mL/m2). The duration of ventilation, ICU stay and hospital stay were lesser in group L (12.75 ± 9.69, 35.95 ± 12.11, 119.10 ± 46.397 vs 23.60 ± 22.03, 51.20 ± 29.92, 140.20 ± 52.65 hours). Conclusions: The incidence of LCOS was lesser in those patients treated with levosimendan, when compared with those treated with milrinone. Cardiac index and stroke volume index were comparable between the two groups. Thus, levosimendan provides a non-inferior alternative to milrinone when used as the primary inotrope following pediatric cardiac surgery.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Piridazinas , Débito Cardíaco , Baixo Débito Cardíaco/prevenção & controle , Cardiotônicos/uso terapêutico , Método Duplo-Cego , Humanos , Hidrazonas/uso terapêutico , Lactente , Milrinona/uso terapêutico , Piridazinas/uso terapêutico , Simendana , Resultado do Tratamento
9.
Indian J Thorac Cardiovasc Surg ; 37(1): 108-111, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33442217

RESUMO

This report describes the use of conventional cardiopulmonary bypass (CPB) circuit beyond the operating room to the critical care unit to provide cardiopulmonary support for patients requiring emergent resuscitation as well as short-term support for rest and recovery of the heart. We modified the CPB circuit and created a closed extra corporeal membrane oxygenation (ECMO) circuit using a conventional oxygenator excluding the venous reservoir. We believe that this technique provides a short-term mechanical support and also acts as a bridge to recovery, or a bridge to decision, in very limited resource settings.

10.
Indian Pediatr ; 57(2): 143-157, 2020 02 15.
Artigo em Inglês | MEDLINE | ID: mdl-32060242

RESUMO

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.


Assuntos
Cardiopatias Congênitas/terapia , Procedimentos Cirúrgicos Cardíacos , Fármacos Cardiovasculares/administração & dosagem , Fármacos Cardiovasculares/uso terapêutico , Criança , Pré-Escolar , Consenso , Humanos , Lactente , Tempo para o Tratamento
11.
World J Pediatr Congenit Heart Surg ; 11(4): NP217-NP220, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30654705

RESUMO

We report a rare case of combined supra- and infracardiac type of total anomalous pulmonary venous connection (mixed-type TAPVC) in a 26-day-old low birth weight (1.9 kg) infant who was admitted with respiratory distress. The child underwent successful surgical repair using a novel surgical technique and is doing well at one year follow-up.


Assuntos
Recém-Nascido de muito Baixo Peso , Veias Pulmonares/cirurgia , Síndrome de Cimitarra/cirurgia , Procedimentos Cirúrgicos Vasculares/métodos , Ecocardiografia , Humanos , Recém-Nascido , Masculino , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Síndrome de Cimitarra/diagnóstico
12.
Indian Heart J ; 71(3): 207-223, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31543193

RESUMO

INTRODUCTION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. OBJECTIVES: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Países em Desenvolvimento , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/tratamento farmacológico , Humanos , Índia , Recém-Nascido , Complicações Pós-Operatórias/prevenção & controle , Fatores de Tempo
13.
Asian Cardiovasc Thorac Ann ; 27(3): 217-220, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30193530

RESUMO

Delayed migration of a ductal occluder device into the aorta after transcatheter closure of a patent ductus arteriosus is extremely rare. We present a case of delayed migration of a ductal occluder into the descending aorta 4 months after its deployment in an 11-year-old girl. Successful surgical removal of the device from the descending aorta and triple-ligation of the ductus arteriosus was performed via a left thoracotomy. The patient made an uneventful recovery.


Assuntos
Aorta , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/instrumentação , Permeabilidade do Canal Arterial/terapia , Migração de Corpo Estranho/etiologia , Dispositivo para Oclusão Septal/efeitos adversos , Aorta/diagnóstico por imagem , Aorta/cirurgia , Aortografia/métodos , Procedimentos Cirúrgicos Cardíacos , Criança , Angiografia por Tomografia Computadorizada , Remoção de Dispositivo/métodos , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/fisiopatologia , Ecocardiografia Doppler em Cores , Feminino , Migração de Corpo Estranho/diagnóstico por imagem , Migração de Corpo Estranho/cirurgia , Humanos , Ligadura , Toracotomia , Resultado do Tratamento
14.
J Indian Assoc Pediatr Surg ; 23(1): 45-47, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29386766

RESUMO

A 7-day-old neonate presented with a large intra-abdominal mass adherent to the hilum of the liver encasing the portal triad. During excision, the portal vein, hepatic artery, and common bile duct were injured. The repair was done promptly and needed massive blood transfusion. Histopathology revealed immature teratoma Grade III. Survival in neonate following total transection of portal triad is rare and has not been reported.

15.
Semin Thorac Cardiovasc Surg ; 28(1): 81-9, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27568142

RESUMO

Review echocardiography and outcomes before and after Ross procedures, including patients undergoing biventricular conversion with Damus-Kaye-Stansel (DKS) takedown. A retrospective review was performed on 62 patients undergoing simple (control group) and complex Ross procedures, including 12 patients who underwent biventricular conversion with Ross operation and DKS takedown (complex). Echocardiography was reviewed preoperatively and at discharge and late follow-up. Kaplan-Meier estimates of patient survival and freedom from reintervention were obtained. In all, 62 patients had a median age of 4.5 years (interquartile range [IQR]: 1-12.5), weight of 16.4kg (IQR: 8-41), and follow-up of 3.8 years (IQR: 1.3-6). The complex DKS takedown group had 2 deaths, no neoaortic valve or root reinterventions, and 3 right ventricular outflow tract (VOT) reinterventions. There were no differences from the control group in left VOT or right VOT reinterventions. Neither group showed differences between pre- and late follow-up aortic root and ascending aorta dimensions, and no correlations were found among preoperative pulmonary valve (PV) size, late aortic regurgitation (AR), aortic root, or ascending aortic Z-scores. Aortic valve size increased from discharge to late follow-up for both groups (P ≤ .05); 90% of patients at late follow-up had mild or less AR with similar distributions in severity between complex and control groups. Severity of late AR showed no correlation with preoperative PV size and is independent of it. The Ross procedure has good short-term results in simple and complex patients and should be considered in those undergoing Ross operation with biventricular conversion and DKS takedown. Moreover, native PV size should not be a contraindication for Ross procedure.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cardiopatias Congênitas/cirurgia , Doenças das Valvas Cardíacas/cirurgia , Valvas Cardíacas/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Adolescente , Criança , Pré-Escolar , Ecocardiografia , Cardiopatias Congênitas/diagnóstico por imagem , Doenças das Valvas Cardíacas/diagnóstico por imagem , Valvas Cardíacas/anormalidades , Valvas Cardíacas/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Estudos Retrospectivos , Adulto Jovem
16.
J Thorac Cardiovasc Surg ; 149(5): 1349-55, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25500103

RESUMO

OBJECTIVE: Aortic root translocation is a promising surgical option for repair of transposition of the great arteries, ventricular septal defect, and pulmonary stenosis. There are little data on the outcomes of this procedure, with no long-term follow-up available. We reviewed our experience with aortic root translocation and the impact of the type of right ventricular outflow tract reconstruction. METHODS: The demographic, procedural, and outcome data were obtained for 32 patients who underwent aortic root translocation from 1997 to 2013 at Boston Children's Hospital. Patients were grouped on the basis of right ventricular outflow tract reconstruction with a valved conduit or a nonvalved anastomosis of the pulmonary artery bifurcation to the right ventricular outflow tract with anterior patch augmentation (transannular patch). RESULTS: The median age was 7.5 months (16 days to 42 years). Twenty-six patients had valved conduits, and 6 patients had transannular patches. There were no significant differences between groups in baseline and operative characteristics. There was 1 early death (transannular patch group). There were no late deaths during a median follow-up of 20.8 months (1 month to 16.5 years). No patients developed late left ventricular outflow tract obstruction. Transcatheter reintervention was required in 14 patients, 9 with valved conduits (34.6%) and 1 with transannular patch (20%, P > .99). Six patients (19.4%) required reoperation, all with a valved conduit (P = .34). CONCLUSIONS: Aortic root translocation can be done with low early and late mortality. There was preserved aortic valve function and no left ventricular outflow tract obstruction at late follow-up. The use of a transannular patch had early outcomes comparable to valved conduits, with a trend for fewer late reoperations.


Assuntos
Anormalidades Múltiplas , Implante de Prótese Vascular/instrumentação , Prótese Vascular , Comunicação Interventricular/cirurgia , Implante de Prótese de Valva Cardíaca/instrumentação , Próteses Valvulares Cardíacas , Estenose da Valva Pulmonar/cirurgia , Transposição dos Grandes Vasos/cirurgia , Adolescente , Adulto , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/mortalidade , Boston , Criança , Pré-Escolar , Comunicação Interventricular/complicações , Comunicação Interventricular/diagnóstico , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/mortalidade , Hospitais Pediátricos , Humanos , Lactente , Recém-Nascido , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/terapia , Desenho de Prótese , Estenose da Valva Pulmonar/complicações , Estenose da Valva Pulmonar/diagnóstico , Estudos Retrospectivos , Fatores de Tempo , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/mortalidade , Resultado do Tratamento , Adulto Jovem
17.
Cardiol Young ; 25(4): 787-9, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24905964

RESUMO

Transposition of the great arteries is a common congenital heart defect causing cyanosis in neonates, occurring in 0.2 per 1000 live births. It has been reported to occur with other associated congenital heart lesions. However, its association with a superior sinus venosus defect and partially anomalous pulmonary venous return has not been reported. We present a neonate with transposition of the great arteries, superior sinus venosus defect with partially anomalous pulmonary venous return that underwent successful complete neonatal repair, and discuss important physiological and anatomic considerations.


Assuntos
Defeitos dos Septos Cardíacos/diagnóstico , Veias Pulmonares/anormalidades , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/diagnóstico , Veia Cava Superior/anormalidades , Ecocardiografia , Humanos , Recém-Nascido , Masculino , Veias Pulmonares/cirurgia , Transposição dos Grandes Vasos/cirurgia , Resultado do Tratamento , Veia Cava Superior/cirurgia
18.
Ann Thorac Surg ; 98(3): 1085-7, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25193192

RESUMO

Congenital mitral valve regurgitation (CMR) is very uncommon in adults and is usually associated with other congenital malformations. Repair of the mitral valve remains the standard of care. Very limited reports are available on minimally invasive surgical approaches in treating CMR. This report represents the first case series of the successful application of robotics in correcting CMR and associated anomalies, including a partial atrioventricular canal defect.


Assuntos
Insuficiência da Valva Mitral/congênito , Insuficiência da Valva Mitral/cirurgia , Robótica , Adolescente , Procedimentos Cirúrgicos Cardíacos/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Indução de Remissão
19.
Ann Thorac Surg ; 98(1): 167-73; discussion 173-4, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24811983

RESUMO

BACKGROUND: Patients with Ebstein malformation (EM) and severe RV dilatation and dysfunction have increased operative risk. Early results with right ventricular unloading utilizing the bidirectional cavopulmonary shunt (BCPS) during repair of EM have been encouraging. We report our experience of the 1.5-ventricle repair strategy for this difficult group of patients. METHODS: Between July 1999 and January 2013, 62 patients with severe EM underwent BCPS at the time of tricuspid repair. Median age was 21.5 years (range, 9 months to 57 years), 51.6% were male, and 72.5% were children. Severe RV dilatation was present in all patients; severe RV dysfunction was present in 72.5% (n=45) and moderate to severe RV dysfunction in 22.5% (n=14). Mean RV systolic pressure was 32.7±0.7 mm Hg and mean PA pressure was 15.6±2.1 mm Hg. Mean preoperative left ventricular ejection fraction (LVEF) was 0.536±0.071; it was less than 40% in 10 patients (16.1%). New York Heart Association class III/IV heart failure was present in 43 patients (69.3%) preoperatively and 20 patients (32.2%) were initially referred for heart transplant evaluation. Prior EM surgery occurred in 35.4% (n=22; 8 prior valve repair, 8 prior valve replacement, Blalock-Taussig shunt in 4, atrial septal defect (ASD) closure in 2). RESULTS: Tricuspid repair was performed in 51.6% (n=32, 5 had re-repair). Bioprosthetic valve replacement was performed in 48.4% (n=30, 8 had rereplacement). The BCPS was a planned procedure in 53 patients (85.5%) because of RV dysfunction; BCPS was added after unsuccessful weaning from bypass in 7 (11.2%), and in the early postoperative period due to hemodynamic instability in 2. Concomitant procedures included ASD closure in 48.3%, maze in 38.7%, and mitral valve repair in 6.4%. Postoperative extracorporeal membrane oxygenation support was needed in 8 patients. Delayed chest closure was performed in 25.8%. Early mortality was 1.6% (n=1). Mean mechanical ventilation time was 69.7 hours. Mean intensive care unit and hospital stays were 5.4±3.5 and 10.7±3.5 days, respectively. Mean follow-up was 3.6±2.6 years (maximum, 12.8 years). Patients (n=10) with low preoperative LVEF (0.362±0.035) improved to 0.517±0.042 postoperatively (p=0.001). There was 1 late death in a patient with cystic fibrosis. Late reintervention was needed in 5 patients (8%). Late follow-up was available in 95% (n=59); all were acyanotic and 88% were in New York Heart Association functional class I/II. CONCLUSIONS: Concomitant BCPS is a useful adjunct in repair of advanced EM with severe RV dilatation and dysfunction. Operation can be performed with low early mortality. Intermediate-term survival and quality of life is good to excellent, and transplantation can be delayed or avoided in the majority.


Assuntos
Anomalia de Ebstein/complicações , Técnica de Fontan/métodos , Insuficiência Cardíaca/cirurgia , Transplante de Coração/métodos , Adolescente , Adulto , Procedimento de Blalock-Taussig , Criança , Pré-Escolar , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/cirurgia , Feminino , Seguimentos , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/mortalidade , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Fatores de Tempo , Resultado do Tratamento , Estados Unidos/epidemiologia , Adulto Jovem
20.
Ann Thorac Surg ; 95(6): 2079-84; discussion 2084-5, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23602064

RESUMO

BACKGROUND: Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. METHODS: From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. RESULTS: Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups. CONCLUSIONS: Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.


Assuntos
Procedimentos Cirúrgicos Cardíacos/mortalidade , Procedimentos Cirúrgicos Cardíacos/métodos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Defeitos dos Septos Cardíacos/cirurgia , Mortalidade Hospitalar , Tetralogia de Fallot/cirurgia , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/mortalidade , Anormalidades Múltiplas/cirurgia , Centros Médicos Acadêmicos , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Pré-Escolar , Estudos de Coortes , Terapia Combinada , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/mortalidade , Ecocardiografia Doppler , Feminino , Seguimentos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/mortalidade , Humanos , Lactente , Estimativa de Kaplan-Meier , Modelos Logísticos , Masculino , Minnesota , Análise Multivariada , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Medição de Risco , Taxa de Sobrevida , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/mortalidade , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
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