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1.
Artigo em Inglês | MEDLINE | ID: mdl-36842797

RESUMO

Medical and surgical advances have allowed single-stage total repair in neonates born with complex congenital heart anomalies and aortic arch obstruction. Nonetheless, total repair might be too complex or high risk in certain neonates with demographic, clinical or morphologic risk factors. Alternative management strategies might offer these neonates better outcomes with superior anatomic repair, shorter hospitalization, reduced morbidity, and improved survival. Alternative initial surgical strategies might include aortic arch repair and pulmonary artery band with or without cardiopulmonary bypass, extracardiac repair only and pulmonary artery band, Norwood operation, and hybrid first-stage palliation; all deferring complex biventricular intra-cardiac repair to later stage. The strategy choice should be personalized to each patient, taking into consideration the morphologic and clinical state, and the existent goals of care.


Assuntos
Doenças da Aorta , Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Procedimentos de Norwood , Recém-Nascido , Humanos , Aorta Torácica/cirurgia , Aorta Torácica/anormalidades , Resultado do Tratamento , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Doenças da Aorta/etiologia , Estudos Retrospectivos
2.
Pediatr Cardiol ; 2022 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-36462027

RESUMO

Treatment of infants with hypoplastic left heart syndrome (HLHS) remains challenging, and those affected remain with significant risks for mortality and morbidity throughout their lifetimes. The maternal-fetal environment (MFE) has been shown to affect outcomes for infants with HLHS after the Norwood procedure. The hybrid procedure, comprised of both catheterization and surgical components, is a less invasive option for initial intervention compared to the Norwood procedure. It is unknown how the MFE impacts outcomes following the hybrid procedure. This is a single-center, retrospective study of infants born with HLHS who underwent hybrid palliation from January 2009 to August 2021. Predictor variables analyzed included fetal, maternal, and postnatal factors. The primary outcome was mortality prior to Stage II palliation. We studied a 144-subject cohort. There was a statistically significant difference in mortality prior to stage II palliation in infants with prematurity, small for gestational age, and aortic atresia subtype (p < 0.001, p = 0.009, and p = 0.008, respectively). There was no difference in mortality associated with maternal diabetes, hypertension, obesity, smoking or illicit drug use, or advanced maternal age. State and national area deprivation index scores were associated with increased risk of mortality in the entire cohort, such that infants born in areas with higher deprivation had a higher incidence of mortality. Several markers of an impaired MFE, including prematurity, small for gestational age, and higher deprivation index scores, are associated with mortality following hybrid palliation. Individual maternal comorbidities were not associated with higher mortality. The MFE may be a target for prenatal counseling and future interventions to improve pregnancy and neonatal outcomes in this population.

3.
Cardiol Young ; 29(3): 375-379, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30724146

RESUMO

IntroductionPulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries, and pulmonary arteries hypoplasia are rare and complex congenital defects that require early interventions to relieve cyanosis and enhance the growth of native pulmonary arteries. The treatment of these patients is still controversial. Surgical techniques require cardiopulmonary bypass which is poorly tolerated by small infants. Percutaneous techniques such as radiofrequency perforation can be challenging. The hybrid technique consists of perventricular stenting of the right ventricle outflow tract through medial sternotomy, to restore native pulmonary flow. METHODS: We retrospectively reviewed the cardiovascular database of our centre in order to analyse our experience in hybrid procedure. We detected six patients who underwent hybrid first approach between November 2007 and December 2015. We report our early results and mid-term outcomes. RESULTS: Median age at the procedure was 26 days, median weight was 3150 g, and median Nakata index was 52 mm2/m2. All procedures were successful except for one: this patient underwent a surgical shunt. No immediate and early deaths or major complications occurred and oxygen saturation levels increased in all the patients. Patients were followed up for a period of 12-103 months, and four of them underwent a procedure of unifocalisation at the mean age of 12.5 months. CONCLUSIONS: We reported data from the largest series of patients who underwent this hybrid procedure. Our experience demonstrated encouraging results to expand the use of this approach to bridge high-risk patients with diminutive pulmonary arteries to a second step of surgical repair.


Assuntos
Anormalidades Múltiplas , Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Ventrículos do Coração/cirurgia , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Stents , Angiografia , Cateterismo Cardíaco/métodos , Feminino , Seguimentos , Comunicação Interventricular/diagnóstico , Humanos , Lactente , Recém-Nascido , Masculino , Atresia Pulmonar/diagnóstico , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
4.
Artigo em Inglês | MEDLINE | ID: mdl-29425520

RESUMO

This article reviews our experience using hybrid stage I palliation in the neonatal period and subsequently with comprehensive stage II palliation for hypoplastic left heart syndrome. Between June 1998 and April 2017, 154 patients with the diagnosis of hypoplastic left heart syndrome and variants underwent a hybrid stage I palliation (bilateral pulmonary artery banding and ductal stenting). One hundred thirty-nine patients could be further univentricularly palliated. One hundred twenty-one patients underwent a comprehensive stage II operation with an operative mortality of 6.6%. The hybrid procedure provides reproducible results with reduced in-hospital, interstage, and long-term mortality and lower rates of aortic arch reinterventions.


Assuntos
Aorta Torácica/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/métodos , Cuidados Paliativos/métodos , Artéria Pulmonar/cirurgia , Stents , Humanos , Recém-Nascido
5.
Cardiol Young ; 28(3): 458-460, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29233199

RESUMO

Survival for hypoplastic left heart syndrome patients following the Norwood procedure is 71-90%. Mortality in patients with Turner's syndrome and hypoplastic left heart syndrome after conventional palliation (Norwood operation) has been reported as high as 80%. This questions the approach of traditional staged palliation. Here, we report a patient with hypoplastic left heart syndrome and Turner's syndrome bridged to orthotopic heart transplantation following a hybrid procedure.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Síndrome de Turner/mortalidade , Síndrome de Turner/cirurgia , Angiografia por Tomografia Computadorizada , Ecocardiografia , Transplante de Coração , Humanos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Lactente , Recém-Nascido , Procedimentos de Norwood/efeitos adversos , Cuidados Paliativos , Diagnóstico Pré-Natal , Resultado do Tratamento , Síndrome de Turner/complicações
6.
Cardiol Young ; 28(2): 252-260, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28889828

RESUMO

Aims The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect. Methods and results From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension. The mean gradient was then measured invasively. Restrictive defect occurred in 11/27 patients (40%). The restrictive group showed three ostium secundum defects (27%) and eight complex morphologies (73%). Conversely, in the non-restrictive group, we observed 11 ostium secundum defects (69%) and five complex morphologies (31%). Early balloon atrioseptostomy was required in three cases. Late restriction occurred in eight patients and was dealt with balloon dilation, stenting, or atrioseptectomy. There was no significant difference between restrictive and non-restrictive groups in terms of early or 12-month survival. CONCLUSIONS: Complex morphologies were more frequently related to restrictiveness. Stenting technique has a crucial role, as the procedure carries a significant risk for stent migration. Effective treatment of restrictive atrial septal defect is related to a better outcome, as it leads to equalisation of survival between patients with and those without restrictive atrial septal defect.


Assuntos
Anormalidades Múltiplas , Cateterismo Cardíaco/métodos , Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interatrial/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Cuidados Paliativos/métodos , Stents , Angiografia , Ecocardiografia Doppler em Cores , Feminino , Seguimentos , Comunicação Interatrial/cirurgia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Risco
7.
Catheter Cardiovasc Interv ; 87(1): E23-9, 2016 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-26198718

RESUMO

For high-risk neonates with hypoplastic left heart syndrome (HLHS) undergoing Norwood operation, the strategy of bilateral pulmonary artery banding and ductal stenting is risky in case of coarctation of the aorta (CoA), often resulting in death. Therefore, we devised a new method of ductal stenting with side-branch cell dilation, which could overcome the constriction of the ductal arch with CoA in two HLHS patients. This is the first report that presents this method and the results. © 2015 Wiley Periodicals, Inc.


Assuntos
Coartação Aórtica/cirurgia , Canal Arterial/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/métodos , Artéria Pulmonar/cirurgia , Stents , Cateterismo Cardíaco , Dilatação , Feminino , Humanos , Lactente , Masculino , Desenho de Prótese
8.
Cardiol Young ; 25(6): 1074-9, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25216030

RESUMO

BACKGROUND: Altered mesenteric perfusion may be a contributor to the development of necrotising enterocolitis in patients with hypoplastic left heart syndrome. The goal of this study was to document mesenteric flow patterns in patients with hypoplastic left heart syndrome pre- and post-hybrid procedure. METHODS: A prospective study on all patients with hypoplatic left heart syndrome undergoing the hybrid procedure was conducted. Doppler ultrasound analysis of the coeliac and superior mesenteric artery was performed. RESULTS: A total of 13 patients were evaluated. There was a significant difference in the coeliac artery effective velocity-time intergral pre- and post-hybrid procedure (8.69±3.84 versus 12.51±4.95 cm, respectively). There were significant differences in the superior mesenteric artery antegrade velocity-time integral pre- and post-hybrid procedure (6.86±2.45 versus 10.52±2.64 cm, respectively) and superior mesenteric artery effective velocity-time integral pre- and post-hybrid procedure (6.22±2.68 versus 9.73±2.73 cm, respectively). There were no significant differences between the coeliac and superior mesenteric artery Doppler indices in the pre-hybrid procedure; there were, however, significant differences in the post-hybrid procedure between coeliac and superior mesenteric artery antegrade velocity-time integral (13.8 2±5.60 versus 10.52±2.64 cm, respectively) and effective velocity-time integral (13.04±4.71 versus 9.73±2.73 cm, respectively). CONCLUSION: Doppler mesenteric indices of perfusion improve in patients with hypoplastic left heart syndrome after the hybrid procedure; however, there appears to be preferential flow to the coeliac artery versus the superior mesenteric artery in these patients post-procedure.


Assuntos
Ecocardiografia Doppler/métodos , Enterocolite Necrosante/epidemiologia , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Artéria Mesentérica Superior/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo , Estudos Transversais , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Masculino , Estudos Prospectivos
9.
World J Pediatr Congenit Heart Surg ; 15(2): 215-223, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38404131

RESUMO

BACKGROUND: Newborns with hypoplastic left heart syndrome (HLHS) who are considered at increased risk for death following Norwood/Sano surgery often undergo hybrid palliation (HP) as initial surgery. We aimed to compile the HP experience in HLHS and its variants and assess the rates of, and risk factors for, death and heart transplantation. METHODS: CINAHL, CINAHL PLUS, PubMed/MEDLINE, and SCOPUS were systematically searched for HP outcome studies of death or heart transplantation in HLHS between 1998 and 2022. Pooled incidence was estimated, and potential risk factors were identified using random-effects meta-analysis and reconstructed time-to-event data from Kaplan-Meier curves. RESULTS: Thirty-three publications were included in our review. Overall, of 1,162 patients 417 died and 57 underwent heart transplantation, resulting in a combined outcome of 40.7%, (474/1,162). There was a trend toward decreasing mortality risk across the stages of palliation. Pooled mortality between HP and comprehensive stage 2 palliation was 25%, after stage 2 up to Fontan palliation was 16%, and 6% post-Fontan. The incidence of death or heart transplantation was higher in high-risk patients-43% died and 10% received heart transplantation. CONCLUSION: Our systematic review and meta-analysis found high rates of death or heart transplantation in HP of HLHS patients between HP and Fontan surgeries. All patients should be closely followed during the initial interstage period, which is associated with the highest hazard. Prospective studies on appropriate patient selection, indications, and / or alternatives, as well as refining HP strategies for managing newborns with HLHS are needed to improve outcomes.


Assuntos
Transplante de Coração , Síndrome do Coração Esquerdo Hipoplásico , Cuidados Paliativos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Transplante de Coração/mortalidade , Cuidados Paliativos/métodos , Recém-Nascido , Procedimentos de Norwood/mortalidade , Fatores de Risco
10.
Eur J Cardiothorac Surg ; 66(1)2024 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-39037957

RESUMO

OBJECTIVES: In patients with borderline left hearts or a severe left ventricular outflow tract obstruction, hybrid palliation can be used to stabilize the patient and postpone biventricular repair (BVR). In this study, we analysed growth of left-sided structures and outcomes of these patients. METHODS: We conducted a retrospective cohort study including patients who received hybrid palliation between January 2010 and September 2023. Echo measurements were collected at hybrid palliation, BVR and last follow-up. Growth of left ventricular structures were analysed. RESULTS: In 38 patients, hybrid palliation was used to promote growth of left ventricular structures. In total, 15 patients received a Ross-Konno/Yasui procedure, while 23 patients received conventional BVR. In patients with a conventional BVR, a significant increase was found in left ventricular volume indexed by body surface area, Z-score of aortic valve and left ventricular outflow tract between hybrid palliation and BVR. Mitral valve Z-score did not increase significantly. After BVR until follow-up, only increase of the aortic valve Z-scores and left ventricular volume indexed by body surface area was found significant. Of all included patients (n = 38), additional surgical procedures were necessary in 8 patients during the interstage period and 15 patients after BVR. Additional catheter interventions were needed in 14 patients in the interstage period and 15 after BVR. Six patients died, with no mortality in the conventional BVR group. CONCLUSIONS: Hybrid palliation as part of a staged BVR is a safe and effective initial step and promotes the growth of left ventricular structures in patients with small left-sided heart structures. Close follow-up is mandatory because extra catheter or surgical interventions are frequently needed.


Assuntos
Ventrículos do Coração , Cuidados Paliativos , Obstrução do Fluxo Ventricular Externo , Humanos , Estudos Retrospectivos , Masculino , Feminino , Ventrículos do Coração/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Cuidados Paliativos/métodos , Obstrução do Fluxo Ventricular Externo/cirurgia , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Lactente , Procedimentos Cirúrgicos Cardíacos/métodos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Ecocardiografia , Resultado do Tratamento
11.
J Clin Med ; 13(14)2024 Jul 20.
Artigo em Inglês | MEDLINE | ID: mdl-39064284

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.

12.
Children (Basel) ; 10(6)2023 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-37371244

RESUMO

Hypoplastic left heart syndrome is a spectrum of complex congenital cardiac defects. Although in borderline cases, biventricular repair is a viable option, in the majority of cases, univentricular palliation is the treatment of choice. Hybrid palliation can be a valid alternative to classic Norwood operation in the neonatal period, especially in selected cases such as high-risk patients or borderline left ventricles. Echocardiography is the main diagnostic modality in this pediatric population, from the fetal diagnosis to the subsequent surgical steps of palliative treatment. Hybrid palliation is performed after birth and is characterized by surgical banding of the pulmonary arteries along with transcatheter stenting of the ductus arteriosus. There are some peculiar aspects of cardiac imaging that characterize this type of palliation, and that should be considered in the different phases before and after the procedure. We aimed to review the current literature about the role of echocardiography in the management of patients with hypoplastic left heart undergoing hybrid palliation.

13.
JTCVS Open ; 13: 330-343, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37063164

RESUMO

Objective: Pulmonary artery reconstruction during comprehensive stage 2 (CS2) procedure can be challenging. Since 2017, we have employed preemptive left pulmonary artery (LPA) stenting. We hypothesized that LPA stenting promotes adequate growth and without compromising Fontan candidacy. Herewith, we report our midterm results. Methods: From 2002 to 2020, 159 patients underwent CS2. Patients were divided as follows: no stent (n = 122; Group 1) and perioperative LPA stent (n = 37; Group 2). Group 2 was subdivided according to unplanned stent (n = 17; Group 2a) or preemptive stent (n = 20; Group 2b). Relevant perioperative data was reviewed. Nonparametric statistics were utilized. Results: Median age and weight at surgery and hospital length of stay after CS2 did not differ between groups. Median cardiopulmonary bypass and crossclamp times were significantly greater in Group 1 (265 vs 243 minutes [P = .021] and 46 vs 26 minutes [P = .008]). In-hospital mortality was similar between Groups 1 and 2 (9.0% vs 18.9%, respectively [P = .1348]). Group 2b demonstrated a superior survival compared to Group 2a (P = .0335) but not Group 1 (P > .9999). Preemptive stenting significantly increased median hilar LPA diameter at CS2 exit angiogram compared with no stenting (P < .0001). Groups 2a and 2b significantly increased the pre-Fontan diameter of the hilar LPA when compared with Group 1 (6.1 and 6.8 vs 5.7 mm, respectively [P < .0001]). A further 120 patients underwent Fontan operation (75%). Median follow-up for Groups 1 and 2 were 7.4 and 3.0 years, respectively. Conclusions: Perioperative LPA stenting during CS2 does not adversely affect pulmonary growth. Preemptive stenting seems advantageous for LPA growth in preparation for Fontan completion.

14.
Children (Basel) ; 10(5)2023 May 11.
Artigo em Inglês | MEDLINE | ID: mdl-37238407

RESUMO

Treatment options for hypoplastic borderline left ventricle (LV) are critically dependent on the development of the LV itself and include different types of univentricular palliation or biventricular repair performed at birth. Since hybrid palliation allows deferring major surgery to 4-6 months, in borderline cases, the decision can be postponed until the LV has expressed its growth potential. We aimed to evaluate anatomic modifications of borderline LV after hybrid palliation. We retrospectively reviewed data from 45 consecutive patients with hypoplastic LV who underwent hybrid palliation at birth between 2011 and 2015. Sixteen patients (mean weight 3.15 Kg) exhibited borderline LV and were considered for potential LV growth. After 5 months, five patients underwent univentricular palliation (Group 1), eight biventricular repairs (Group 2) and three died before surgery. Echocardiograms of Groups 1 and 2 were reviewed, comparing LV structures at birth and after 5 months. Although, at birth, all LV measurements were far below the normal limits, after 5 months, LV mass in Group 2 was almost normal, while in Group 1, no growth was evident. However, aortic root diameter and long axis ratio were significantly higher in Group 2 already at birth. Hybrid palliation can be positively considered as a "bridge-to-decision" for borderline LV. Echocardiography plays a key role in monitoring the growth of borderline LV.

15.
J Thorac Cardiovasc Surg ; 166(5): 1300-1313.e2, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37164059

RESUMO

OBJECTIVE: To compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy). METHODS: From 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups. Overall survival between the 2 groups was adjusted by applying balancing scores to nonparametric estimates. RESULTS: Compared with the nonhybrid management group, infants who underwent hybrid palliation had lower birth weight, smaller gestational age, and higher prevalence of in-utero interventions, noncardiac comorbidities, preoperative mechanical ventilation, absent interatrial communication, and moderate or severe mitral valve stenosis (all P values < .03). Unadjusted 12-year survival after hybrid palliation and nonhybrid management, was 55% versus 69%, respectively. After matching, 12-year survival after hybrid palliation versus nonhybrid management was 58% versus 63%, respectively (P = .37). Among matched infants born weighing <2.5 kg, 2-year survival after hybrid palliation versus nonhybrid management was 37% versus 51%, respectively (P = .22). CONCLUSIONS: Infants born with critical left heart obstruction who undergo hybrid palliation have more high-risk characteristics and anatomy versus infants who undergo nonhybrid management. Nonetheless, after adjustment, there was no significant difference in 12-year survival after hybrid palliation versus nonhybrid management. Mortality remains high, and hybrid palliation confers no survival advantage, even for lower-birth-weight infants.

16.
J Thorac Cardiovasc Surg ; 165(1): 287-298.e4, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-35599210

RESUMO

OBJECTIVE: Prematurity, low birth weight, genetic syndromes, extracardiac conditions, and secondary cardiac lesions are considered high-risk conditions associated with mortality after stage 1 palliation. We report the impact of these conditions on outcomes from a prospective multicenter improvement collaborative. METHODS: The National Pediatric Cardiology Quality Improvement Collaborative Phase II registry was queried. Comorbid conditions were categorized and quantified to determine the cumulative burden of high-risk diagnoses on survival to the first birthday. Logistic regression was applied to evaluate factors associated with mortality. RESULTS: Of the 1421 participants, 40% (575) had at least 1 high-risk condition. The aggregate high-risk group had lower survival to the first birthday compared with standard risk (76.2% vs 88.1%, P < .001). Presence of a single high-risk diagnosis was not associated with reduced survival to the first birthday (odds ratio, 0.71; confidence interval, 0.49-1.02, P = .066). Incremental increases in high-risk diagnoses were associated with reduced survival to first birthday (odds ratio, 0.23; confidence interval, 0.15-0.36, P < .001) for 2 and 0.17 (confidence interval, 0.10-0.30, P < .001) for 3 to 5 high-risk diagnoses. Additional analysis that included prestage 1 palliation characteristics and stage 1 palliation perioperative variables identified multiple high-risk diagnoses, poststage 1 palliation extracorporeal membrane oxygenation support (odds ratio, 0.14; confidence interval, 0.10-0.22, P < .001), and cardiac reoperation (odds ratio, 0.66; confidence interval, 0.45-0.98, P = .037) to be associated with reduced survival odds to the first birthday. CONCLUSIONS: The presence of 1 high-risk diagnostic category was not associated with decreased survival at 1 year. Cumulative diagnoses across multiple high-risk diagnostic categories were associated with decreased odds of survival. Further patient accrual is needed to evaluate the impact of specific comorbid conditions within the broader high-risk categories.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Criança , Humanos , Procedimentos de Norwood/efeitos adversos , Estudos Prospectivos , Estudos Retrospectivos , Cuidados Paliativos , Fatores de Risco , Resultado do Tratamento
17.
J Thorac Cardiovasc Surg ; 166(3): 943-954.e1, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-36804212

RESUMO

OBJECTIVE: To determine patient characteristics and outcomes after Norwood versus comprehensive stage II (COMPSII) for infants with critical left heart obstruction who had prior hybrid palliation (bilateral pulmonary artery banding ± ductal stent). METHODS: From 23 Congenital Heart Surgeons' Society institutions (2005-2020), 138 infants underwent hybrid palliation followed by either Norwood (n = 73, 53%) or COMPSII (n = 65). Baseline characteristics were compared between Norwood and COMPSII groups. Parametric hazard model with competing risk methodology was used to determine risk and factors associated with outcomes of Fontan, transplantation, or death. RESULTS: Infants who underwent Norwood versus COMPSII had a higher prevalence of prematurity (26% vs 14%, P = .08), lower birth weight (median 2.8 vs 3.2 kg, P < .01) and less frequent ductal stenting (37% vs 99%; P < .01). Norwood was performed at a median age of 44 days and median weight of 3.5 kg, versus COMPSII at 162 days and 6.0 kg (both P < .01). Median follow-up was 6.5 years. At 5 years after Norwood and COMPSII, respectively; 50% versus 68% had Fontan (P = .16), 3% versus 5% had transplantation (P = .70), 40% versus 15% died (P = .10), and 7% versus 11% are alive without transition, respectively. For factors associated with either mortality or Fontan, only preoperative mechanical ventilation occurred more frequently in the Norwood group. CONCLUSIONS: Higher prevalence of prematurity, lower birth weight, and other patient-related characteristics in the Norwood versus COMPSII groups may influence differences in outcomes that were not statistically significant for this limited risk-adjusted cohort. The clinical decision regarding Norwood versus COMPSII after initial hybrid palliation remains challenging.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Lactente , Humanos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Peso ao Nascer , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Resultado do Tratamento , Bloqueio Cardíaco , Cuidados Paliativos/métodos , Estudos Retrospectivos , Fatores de Risco
18.
Interact Cardiovasc Thorac Surg ; 34(4): 613-615, 2022 03 31.
Artigo em Inglês | MEDLINE | ID: mdl-34888682

RESUMO

Aortic reconstruction at the time of the comprehensive stage II (CSII) procedure can be complicated by compression within the aortopulmonary space resulting in airway or pulmonary artery narrowing. We describe our experience with 2 patients with hypoplastic left heart syndrome and pulmonary artery stenosis after the CSII procedure. Both patients underwent an aortic extension with a Hemashield interposition graft to open up the aortopulmonary space. The patients were discharged from the hospital. In all cases the aortopulmonary space was enlarged, and the pulmonary arteries and airway were free from compression. Aortic extension is an option to be considered in children with pulmonary artery compression who previously had a CSII procedure.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Aorta/diagnóstico por imagem , Aorta/cirurgia , Criança , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Cuidados Paliativos/métodos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares
19.
World J Pediatr Congenit Heart Surg ; 13(5): 588-592, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-36053094

RESUMO

The Giessen hybrid strategy is used for initial palliation of HLHS and variants when the intent is to pursue further staged palliative reconstruction toward Fontan circulation. It is also used for initial palliation of HLHC and other anomalies with potential for eventual biventricular repair. From June 1998 - October 2021, 197 patients with HLHS and related variants underwent hybrid stage 1. Follow-up is complete (median 8.3 [range 0-23.3] years). Operative mortality for hybrid stage I was 3.6% (7/197); reduced to 2.8% since 2010 (4/141). Interstage mortality was 4.1% (8/197). Operative mortality for comprehensive stage II was 5.8% (10/172), and since 2010 was 1.8% (2/113 patients). Fontan completion has been accomplished in 117 patients, and 33 are still awaiting stage III. Twelve patients underwent heart transplantation. Over 23 years, overall survival is 77.7% and transplant-free survival is 73.6%.


Assuntos
Técnica de Fontan , Transplante de Coração , Síndrome do Coração Esquerdo Hipoplásico , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Lactente , Cuidados Paliativos , Estudos Retrospectivos , Resultado do Tratamento
20.
Indian J Thorac Cardiovasc Surg ; 37(3): 345-347, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33967429

RESUMO

We report a unique experience with stage 1 palliation of hypoplastic left heart syndrome (HLHS) using the hybrid approach. The tortuous anatomy of the ductus arteriosus precluded stenting. Persistent ductal patency and ability to support the systemic circulation adequately after prolonged prostaglandin E1 (PGE1) infusion and its subsequent discontinuation presented us with a novel approach to hybrid palliation of hypoplastic left heart syndrome. Our experience offers a tempting approach, but it should not be considered a standard alternative management option to stage I hybrid palliation based on a single case report. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12055-020-01088-8.

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