RESUMO
Background/Objectives: Hybrid palliation (HP) procedures for hypoplastic left heart syndrome (HLHS) are increasing. Our objective was to compare mortality and morbidity following HP and NP (Norwood palliation) procedures. Methods: Systematic review and meta-analysis of HLHS patients of peer-reviewed literature between 2000 and 2023. Mortality and/or heart transplantation in HP versus NP in the neonatal period, interstage period, and at 1, 3 and 5 years of age, and morbidity including completion of Stage II and Stage III palliation, unexpected interventions, pulmonary artery pressures, right ventricle function, neurodevelopmental outcomes and length of hospital stay were evaluated. Results: Twenty-one (meta-analysis: 16; qualitative synthesis: 5) studies evaluating 1182 HLHS patients included. HP patients had higher interstage mortality (RR = 1.61; 95% CI: 1.10-2.33; p = 0.01) and 1-year mortality (RR = 1.22; 95% CI: 1.03-1.43; p = 0.02) compared to NP patients without differences in 3- and 5-years mortality. HP procedure in high-risk HLHS patients had lower mortality (RR = 0.48; 95% CI: 0.27-0.87; p = 0.01) only in the neonatal period. HP patients underwent fewer Stage II (RR = 0.90; 95% CI: 0.81-1.00; p = 0.05) and Stage III palliation (RR = 0.78; 95% CI: 0.69-0.90; p < 0.01), had more unplanned interventions (RR = 3.38; 95% CI: 2.04-5.59; p < 0.01), and longer hospital stay after Stage I palliation (weighted mean difference = 12.88; 95% CI: 1.15-24.62; p = 0.03) compared to NP patients. Conclusions: Our study reveals that HP, compared to NP for HLHS, is associated with increased morbidity risk without an improved survival rate.
RESUMO
BACKGROUND: Congenital pulmonary airway malformations (CPAMs) are a heterogenous collection of congenital lung malformations, often diagnosed prenatally. The Stocker Type III CPAM is a rare CPAM sub-type, and, when large, may be associated with hydrops. Furthermore, reports of CPAM management which may include surgical resection in extreme preterm infants are limited. CASE PRESENTATION: We report a case of a female neonate born at 28 weeks of gestation with severe respiratory distress and diffuse pulmonary opacification on the right concerning for a large congenital lung lesion. This lesion was not detected on routine antenatal imaging, and she did not have clinical findings of associated hydrops. Her respiratory status improved dramatically after surgical resection of a mass at 12 day of age. The mass was consistent pathologically with a Stocker Type III CPAM. Lung expansion showed subsequent improvement at 16 months of age. CONCLUSIONS: Our case describes a preterm neonate with severe respiratory distress that was found postnatally to have a large, unilateral congenital lung lesion despite a normal prenatal ultrasound. Additionally, this lesion required excision early in life due to severity of respiratory compromise. This case highlights that rare congenital lung lesions, like this rare sub-type of CPAM, should remain a diagnostic consideration in neonates with severe respiratory distress. Early lung resection for CPAM in preterm infants is not well described and the favorable outcomes of this case help expand perspectives on potential management strategies.
Assuntos
Malformação Adenomatoide Cística Congênita do Pulmão , Síndrome do Desconforto Respiratório , Lactente , Feminino , Recém-Nascido , Humanos , Gravidez , Recém-Nascido Prematuro , Malformação Adenomatoide Cística Congênita do Pulmão/complicações , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Pulmão/diagnóstico por imagem , Pulmão/cirurgia , Pulmão/anormalidades , Dispneia , Síndrome do Desconforto Respiratório/patologia , EdemaRESUMO
Background: Bidirectional Glenn shunt (BDG) failure carries high morbidity and mortality but the clinical factors associated with failure and the optimal management strategy are understudied. Methods: A total of 217 patients undergoing BDG at our institution between 1989 and 2020 were retrospectively reviewed and categorized as success or failure. Failure was defined as the need for reoperation (BDG takedown, reoperation for correction of cardiac defect, and/or transplantation) at any time postoperatively; operative mortality (death attributable to BDG malfunction occurring during the index hospitalization for BDG or within 30 days of discharge); or late mortality (death directly attributable to BDG malfunction occurring prior to Fontan or next-stage palliation). Univariate and binary logistic regression analyses were performed. Results: BDG failure occurred in 14 (6.5%) patients. Univariate predictors were: hypoplastic left heart syndrome (P = .037), right ventricular (RV) dominance (P = .010), greater pre-BDG pulmonary vascular resistance (PVR) (P = .012), concomitant atrioventricular valve repair (P = .020), prolonged pleural drainage (P = .001), intensive care unit (P<.001) and hospital (P = .002) stays, and extracorporeal membrane oxygenation (ECMO) requirement (P<.001). Multivariate predictors were: RV dominance (P = .002), greater PVR (P = .041), ICU (P<.001) and hospital (P = .020) stays, and need for ECMO (P<.001). As many as 10 of 14 (71%) patients with BDG failure died. Reoperation was performed for 10 patients with BDG failure. Five reoperation patients survived until discharge, with four patients alive at last follow-up (mean 7.9 years). Survivors underwent reoperation earlier than nonsurvivors (36 vs. 94 days). Conclusions: BDG failure carries high mortality, but preoperative predictors and postoperative indicators of failure exist. Early BDG takedown and insertion of aorta-pulmonary shunt may allow survival.
Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Reoperação , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
The objective of this study was to determine medium-term morbidity and mortality of patients who have undergone device closure of an extracardiac Fontan fenestration with an Amplatzer Vascular Plug II (AVPII) or Septal Occluder (ASO). A secondary objective was to compare medium-term morbidity and mortality between these patients and other fenestrated Fontan patients. A retrospective chart review was performed on patients who underwent an extracardiac fenestrated Fontan procedure between 1992 and 2015 at Cardinal Glennon Children's Medical Center. Procedural and follow-up data were obtained and compared between those who underwent fenestration closure and those who did not. Additional outcome measures included whether the fenestration had spontaneously closed, morbidity and mortality, oxygen saturations, and hemodynamics pre- and post-closure. Fifty-nine of 118 patients (50%) with a fenestrated Fontan underwent 60 device closures of the fenestration. Thirty-two (53%) of these were with the AVPII and 28 (47%) with the ASO. There was one device embolization. At a median follow-up of 3.9 years, five patients suffered morbidity, including 2 with arrhythmias, 1 with plastic bronchitis, 1 with protein losing enteropathy, and 1 with stroke. There were no cardiopulmonary deaths in this group. Twenty-three of 118 patients (19%) had spontaneous closure. There was no difference in morbidity and mortality between patients who underwent percutaneous fenestration closure and those who either had spontaneous closure or a persistently patent fenestration. Device closure of Fontan fenestrations is a safe and effective procedure with minimal morbidity and mortality comparable to other patients with fenestrations.
Assuntos
Embolização Terapêutica/instrumentação , Técnica de Fontan/métodos , Adolescente , Cateterismo Cardíaco/métodos , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Seguimentos , Técnica de Fontan/efeitos adversos , Humanos , Masculino , Estudos Retrospectivos , Dispositivo para Oclusão SeptalRESUMO
Anomalous origin of the right coronary artery from the pulmonary artery is an extremely rare condition with an uncertain natural history. Here, we present an otherwise healthy active duty service member who was found to have anomalous right coronary artery arising from the pulmonary artery as an incidental discovery during a workup for possible pulmonary embolus. We describe a preoperative workup which showed dilation of the coronaries and evidence of ischemia. Operative technique for restoration of a two-vessel coronary system is described. Postoperative imaging demonstrated reduction in the coronary dilation and resolution of ischemia.
Assuntos
Anomalias dos Vasos Coronários/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Artéria Pulmonar/cirurgia , Angiografia Coronária , Anomalias dos Vasos Coronários/diagnóstico por imagem , Testes Diagnósticos de Rotina , Humanos , Achados Incidentais , Masculino , Pessoa de Meia-Idade , Militares , Período Pós-Operatório , Período Pré-Operatório , Artéria Pulmonar/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagemRESUMO
An increased incidence of CHD has been noted in twin gestations and in infants conceived using assisted reproductive technologies. However, CHD in these populations remains understudied and the mechanisms underlying these phenomena remain unclear. We present the case of twins conceived via in vitro fertilisation both with Tetralogy of Fallot and additional cardiac and extracardiac malformations.