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1.
World J Pediatr Congenit Heart Surg ; 13(5): 609-614, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-36053092

RESUMO

Hypoplastic left heart syndrome (HLHS) is possibly the most challenging congenital heart defect to confront in any setting. The highly specialized infrastructure and resources needed to treat HLHS is not available in many low-resource settings. However, low-resource settings must not be assumed to be synonymous with low- and middle-income countries as national income is not necessarily indicative of a country's prioritization of healthcare resources. Besides, a low-resource setting may be institution-specific as well as country-specific. We have stratified institutional capabilities for addressing the requirements of treatment for HLHS into five levels based on the capacity for diagnosis, intervention, and post-discharge monitoring. Depending on institutional capabilities, children born with HLHS in low-resource settings experience a spectrum of outcomes ranging from death without diagnosis to the hybrid or Norwood stage 1 palliation. The decision-making is ethically challenging when resources are scarce and economic efficiency must be considered in the context of distributive justice. Even in settings that would be classified as resource-rich where survival after surgery and quality of life afterward keep improving, not every parent would choose surgical intervention for their hypothetical child with HLHS.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Assistência ao Convalescente , Criança , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Cuidados Paliativos , Alta do Paciente , Qualidade de Vida , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
2.
Pediatr Cardiol ; 42(6): 1394-1404, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33987707

RESUMO

Overall survival of patients with hypoplastic left heart syndrome (HLHS) has shown continued improvement. Right ventricular (RV) dysfunction, in the long term, adversely affects prognosis in these patients. This study examines changes in echocardiographic markers of RV function in a longitudinal cohort. We retrospectively reviewed patients with HLHS managed at our institution from 7/1994 to 1/2016. Follow-up included surgical and clinical data, and echocardiographic measures. Measures of RV function preceding and following all three stages of single ventricular palliation were collected. Freedom from transplant-free survival was assessed by Kaplan-Meier analysis. Multivariable associations with time to death or transplant were explored using the Cox proportional hazards model. A total of 120 patients with HLHS were identified. Norwood operation was performed in all patients. The probability of survival for the cohort was 71 ± 4.4%, 69 ± 4.5% and 66 ± 4.7% at 1, 2 and 5 years respectively after stage I Norwood operation. RV fractional area change (FAC), compared to post-Norwood was decreased at all subsequent stages with the greatest change noted post-superior cavo-pulmonary shunt from 40.7 ± 9.3% to 31.1 ± 8.3% (p < 0.001). Similarly, tricuspid valve annular systolic excursion (TAPSE) Z-score declined from -2.9 ± 1.3 to -9.7 ± 1.3 (p < 0.001) with a decrement at every stage of evaluation. In comparison to patients with post-Norwood RV FAC >35% and TAPSE Z-score > -5, patients with RV FAC ≤ 35% and TAPSE Z-score ≤ -5 had a significantly lower transplant-free survival (p < 0.0001). In patients with HLHS undergoing staged palliation, decrement in RV function manifests longitudinally. Post-Norwood RV FAC and TAPSE Z-score appear to be early markers of poor outcome in this population.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Função Ventricular Direita/fisiologia , Ecocardiografia , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Masculino , Procedimentos de Norwood/métodos , Prognóstico , Estudos Retrospectivos , Sístole
4.
Congenit Heart Dis ; 11(6): 727-732, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27320439

RESUMO

OBJECTIVES: Caring for children with congenital heart disease places significant stress on caregivers. Minimal data exist evaluating stress levels in caregivers of children with hypoplastic left heart syndrome (HLHS). The goal of this study was to obtain baseline stress scores for caregivers of children with HLHS and determine if associations exist between scores and specific caregiver factors. STUDY DESIGN: A cross-sectional study using a web-based survey targeted towards caregivers of children with HLHS was performed. Baseline demographics of the caregiver and child with HLHS were obtained. Caregivers completed three validated questionnaires including the Pediatric Quality of Life Inventory (PedsQL), Parenting Stress Index- Short Form (PSI-SF) and the Pediatric Inventory for Parents (PIP). RESULTS: Four hundred fifty-nine caregivers completed at least one questionnaire. PSI-SF total score was 80.5 ± 23.1 (> 86 = significant stress), PIP frequency total score was 119.0 ± 37.2, and PIP difficulty total score was 118.1 ± 35.7. Lower quality of life was significantly correlated with higher scores on the PSI-SF (r = -0.6), the presence of a developmental issue in the child (r = 0.3) as well as higher scores on the PIP frequency (r = -0.5) and difficulty scales (r = 0.4). Other demographic values for the caregiver and child did not significantly correlate with PSI or PIP total scores. CONCLUSIONS: Anxiety/stress scores of caregivers with children with HLHS are correlated with how well the child is perceived to be doing physically and developmentally by the caregivers. Caregivers with physical and/or developmental concerns may need added psychosocial support.


Assuntos
Ansiedade/etiologia , Cuidadores/psicologia , Síndrome do Coração Esquerdo Hipoplásico/terapia , Qualidade de Vida , Estresse Psicológico/etiologia , Adaptação Psicológica , Adulto , Ansiedade/diagnóstico , Ansiedade/psicologia , Efeitos Psicossociais da Doença , Estudos Transversais , Feminino , Inquéritos Epidemiológicos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/psicologia , Masculino , Saúde Mental , Estudos Prospectivos , Fatores de Risco , Estresse Psicológico/diagnóstico , Estresse Psicológico/psicologia
5.
Biomed Res Int ; 2016: 2616729, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26981527

RESUMO

Aims. To detect anatomical and intrinsic histopathological features of the ascending aorta and left ventricular (LV) myocardium and evaluate right ventricular (RV) function in fetuses with hypoplastic left heart syndrome (HLHS). Methods. Twenty-five fetuses diagnosed with HLHS were followed up in the antenatal and postpartum periods. 12 necropsy heart specimens were analyzed for morphological and histological changes. Results. Prenatal echocardiography and pathologic anatomy displayed the typical characteristics of HLHS as a severe underdevelopment of the LV in the form of mitral stenosis or atresia or as aortic atresia or stenosis, with a decreased ratio of aortic diameter to pulmonary artery diameter (median of 0.49 with a range of 0.24 to 0.69, p ≤ 0.001) and a higher ratio of RV diameter to LV diameter (median of 2.44 with a range of 1.33 to 6.25, p ≤ 0.001). The RV volume, stroke volume, and cardiac output in HLHS fetuses were increased compared with the gestational age-matched normal controls (p < 0.01). Histological changes in the 12 HLHS specimens included LV myocardial fibrosis, aortic elastic fragmentation, and fibrosis. Conclusions. In addition to severe anatomical deformity, distinct histological abnormalities in the LV myocardium and aortic wall were identified in the fetuses with HLHS. RV function damage may be potentially exists.


Assuntos
Aorta/fisiopatologia , Ventrículos do Coração/fisiopatologia , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Miocárdio/patologia , Ecocardiografia , Feminino , Feto/patologia , Idade Gestacional , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Período Pós-Parto , Gravidez , Ultrassonografia Pré-Natal
6.
Cardiol Young ; 25(6): 1214-7, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25275342

RESUMO

Using phase-contrast MRI in a foetus with borderline left ventricular hypoplasia at 37 weeks' gestation we showed an increase in pulmonary blood flow during maternal hyperoxygenation. The associated increase in venous return to the left atrium, however, resulted in reversal of the atrial shunt, with no improvement in left ventricular output. The child initially underwent single ventricle palliation with a neonatal hybrid procedure, but following postnatal growth of the left ventricle tolerated conversion to a biventricular circulation at 5 months of age. We conclude that when there is significant restriction of filling or outflow obstruction across the left heart, neither prenatal nor postnatal acute pulmonary vasodilation can augment left ventricular output enough to support a biventricular circulation. Chronic pulmonary vasodilation may stimulate the growth of the left-sided structures allowing biventricular repair, raising the intriguing question of whether chronic maternal oxygen therapy might obviate the need for neonatal single ventricle pallation in the setting of borderline left ventricular hypoplasia.


Assuntos
Átrios do Coração/anormalidades , Ventrículos do Coração/anormalidades , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Artéria Pulmonar/cirurgia , Ecocardiografia , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Stents
7.
J Thorac Cardiovasc Surg ; 149(1): 195-201, 202.e1, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25266878

RESUMO

OBJECTIVE: Hybrid palliation is an alternative to Norwood stage 1 for the initial management of hypoplastic left heart syndrome. Contemporary multicenter hybrid use and institutional/patient factors associated with hybrid use relative to the Norwood have not been evaluated. We describe hybrid use in relation to institutional volume, patient factors, and short-term outcomes. METHODS: Infants aged 60 days or less listed in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2012) undergoing initial palliation of hypoplastic left heart syndrome were included. Annual institutional hybrid use rates were calculated: [hybrid procedures/(Norwood + hybrid + transplant procedures)]. In-hospital outcomes for primary hybrid and primary Norwood were compared and stratified by high (defined as ≥50%) versus low (defined as ≤10%) institutional hybrid use. RESULTS: Of 1728 patients (100 centers), most (n = 1496, 87%) underwent an index Norwood; 232 patients (13%) underwent an index hybrid procedure. Preoperative patient risk factors were more prevalent in patients undergoing the hybrid procedure. Only 13 of 100 institutions were high hybrid users, and these tended to have lower annual hypoplastic left heart syndrome index case volume. Unadjusted in-hospital mortality was higher for the hybrid compared with the Norwood procedure (30% vs 16%; P < .001). In-hospital mortality for the hybrid procedure was not associated with hybrid use (26% among institutions with low use vs 28% among institutions with high use). However, centers with high hybrid use had higher mortality after the Norwood (43%) compared with centers with low hybrid use (16%). CONCLUSIONS: Few centers currently select the hybrid procedure for most infants with hypoplastic left heart syndrome. Although unadjusted in-hospital hybrid mortality is higher than Norwood mortality, potential risk factors are more prevalent among hybrid cases. Institutions with higher hybrid use have lower hypoplastic left heart syndrome case volume and higher Norwood mortality.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/tendências , Cuidados Paliativos/tendências , Artéria Pulmonar/cirurgia , Canadá , Bases de Dados Factuais , Feminino , Transplante de Coração/tendências , Mortalidade Hospitalar/tendências , Hospitais com Alto Volume de Atendimentos/tendências , Hospitais com Baixo Volume de Atendimentos/tendências , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Lactente , Mortalidade Infantil/tendências , Recém-Nascido , Masculino , Procedimentos de Norwood/efeitos adversos , Procedimentos de Norwood/mortalidade , Artéria Pulmonar/fisiopatologia , Fatores de Risco , Sociedades Médicas , Fatores de Tempo , Resultado do Tratamento , Estados Unidos
8.
J Thorac Cardiovasc Surg ; 149(1): 205-10.e1, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25227701

RESUMO

OBJECTIVES: Our aim was to examine the effects of surgical timing on major morbidity, mortality, and total hospital reimbursement for late preterm and term infants with hypoplastic left heart syndrome (HLHS) undergoing stage 1 palliation within the first 2 weeks of life. METHODS: We conducted a retrospective cohort study of infants aged ≥35 weeks gestation, with HLHS, admitted to our institution at age ≤5 days, between January 1, 2003, and January 1, 2013. Children with other cardiac abnormalities or other major comorbid conditions were excluded. Univariable and multivariable analyses were performed to determine the association between age at stage 1 palliation and major morbidity, mortality, and hospital reimbursement. RESULTS: One hundred thirty-four children met inclusion criteria. Mortality was 7.5% (n = 10). Forty-three percent (n = 58) experienced major morbidity. Median costs were $97,000, in 2013 dollars (interquartile range, $72,000-$151,000). Median age at operation was 5 days (interquartile range, 3-7 days; full range, 1-14 days). All deaths occurred in patients operated on between 4 and 8 days of life. For every day later that surgery was performed, the odds of major morbidity rose by 15.7% (95% confidence interval, 2.5%-30.7%; P = .018) and costs rose by 4.7% (95% confidence interval, 0.9%-8.2%; P < .014). CONCLUSIONS: Delay of stage 1 palliation for neonates with HLHS is associated with increased morbidity and health care costs, even within the first 2 weeks of life.


Assuntos
Redução de Custos , Custos Hospitalares , Síndrome do Coração Esquerdo Hipoplásico/economia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Cuidados Paliativos/economia , Tempo para o Tratamento/economia , Distribuição de Qui-Quadrado , Feminino , Idade Gestacional , Mortalidade Hospitalar , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Lactente , Mortalidade Infantil , Recém-Nascido , Recém-Nascido Prematuro , Reembolso de Seguro de Saúde , Modelos Lineares , Modelos Logísticos , Masculino , Análise Multivariada , Cidade de Nova Iorque , Razão de Chances , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
9.
Heart Surg Forum ; 16(6): E313-8, 2013 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24370799

RESUMO

BACKGROUND: There has been a notable improvement in the outcome of stage 1 palliation for hypoplastic left heart syndrome (HLHS) in recent years. Nevertheless, developing a new Norwood program requires a steep learning curve, especially in emerging economies where rapid population growth brings a high volume of patients but, on the other hand, resources are limited. In this paper we aimed to summarize the initial results of a single center. METHODS: Hospital records of 21 patients were reviewed for all patients having a stage 1 palliation procedure for HLHS between May 2011 and May 2013. There were 13 male (62%) and 8 female (38%) patients. Median age was 14 days (range, 4-74 days) and median weight was 3030 g (2600-3900 g). HLHS was defined as mitral or aortic stenosis or atresia (or both) in the presence of normally related great vessels and a hypoplastic left ventricle. Transthoracic echocardiography was the diagnostic modality used in all patients. All procedures but one were performed using an antegrade selective cerebral perfusion method and moderate hypothermia. Cerebral perfusion was monitored with cerebral oximetry in all patients. Modified ultrafiltration was routinely used in all patients. RESULTS: Overall hospital mortality was 47.6% (n = 10). Mortality rates considerably decreased from the first year to second year (69% and 12.5% respectively). No risk factors were identified for mortality. CONCLUSIONS: Surgical palliation of neonates with hypoplastic left heart syndrome continues to be a challenge. To decrease the overall mortality nationwide and improve outcomes, a referral center with a dedicated team is necessary in emerging economies.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/terapia , Curva de Aprendizado , Cuidados Paliativos/organização & administração , Procedimentos Cirúrgicos Profiláticos/economia , Procedimentos Cirúrgicos Cardíacos/economia , Países em Desenvolvimento , Feminino , Mortalidade Hospitalar , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/economia , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Lactente , Recém-Nascido , Masculino , Cuidados Paliativos/métodos , Desenvolvimento de Programas , Procedimentos Cirúrgicos Profiláticos/métodos , Resultado do Tratamento , Turquia
10.
Am J Cardiol ; 111(12): 1792-9, 2013 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-23538019

RESUMO

Staged surgical palliation has revolutionized the care of patients with hypoplastic left heart syndrome (HLHS), although the outcomes of survival and cost at a national level remain unclear. This study sought to evaluate (1) trends in HLHS surgical outcomes including in-hospital mortality, length of stay (LOS), and cost, and (2) patient and hospital risk factors associated with these outcomes. Hospitalizations for patients with HLHS, including stage I, II, and III palliations, were analyzed using the Kids' Inpatient Database from 2000 through 2009. Trends in mortality, LOS, and cost were analyzed and chi-squared tests were used to test association between categorical variables. Patient and hospital characteristics associated with death were analyzed using logistic regression and associations with LOS were analyzed using ordinary least squared regression. There were 16,923 hospital admissions in patients with HLHS of which 5,672 (34%) included surgical intervention. Total (3,201-5,102) and surgery-specific admissions (1,165-1,618) increased from 2000 to 2009. Mortality decreased 14% per year in stage III palliations (odds ratio [OR] 0.86; 95% confidence interval [CI]: 0.79-0.94) and 6% per year for stage I palliations (OR 0.94; 95% CI 0.90-0.99) but not for stage II palliations (OR 1.01; 95% CI; 0.89-1.14). Length of stay increased for stage I and II palliations; however, per-patient hospital cost decreased in 2009. In conclusion, recent decrease in per patient cost for staged surgical palliation for HLHS has correlated temporally with improved mortality.


Assuntos
Custos de Cuidados de Saúde , Mortalidade Hospitalar/tendências , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Pacientes Internados/estatística & dados numéricos , Tempo de Internação/estatística & dados numéricos , Cuidados Paliativos/economia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/economia , Lactente , Recém-Nascido , Tempo de Internação/economia , Masculino , Prontuários Médicos , Cuidados Paliativos/estatística & dados numéricos , Cuidados Paliativos/tendências , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Estados Unidos/epidemiologia
12.
Cardiol Young ; 21(1): 116-9, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-20977828

RESUMO

Precise lung perfusion quantification is essential for evaluation of patients with hemi-Fontan surgery. It is possible for two-dimensional cardiac magnetic resonance phase contrast flow (two-dimensional flow) to evaluate non-invasively the systemic-to-pulmonary collateral blood flow. This case report intends to illustrate the benefit of four-dimensional flow over the current two-dimensional flow in the comprehensive assessment of hemi-Fontan patients.


Assuntos
Velocidade do Fluxo Sanguíneo/fisiologia , Circulação Colateral/fisiologia , Doenças das Valvas Cardíacas/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Processamento de Imagem Assistida por Computador , Imagem Cinética por Ressonância Magnética/métodos , Valva Mitral/anormalidades , Pré-Escolar , Diagnóstico Diferencial , Doenças das Valvas Cardíacas/congênito , Doenças das Valvas Cardíacas/fisiopatologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Masculino , Veias Pulmonares/fisiopatologia , Veias Cavas/fisiopatologia
13.
J Thorac Cardiovasc Surg ; 136(6): 1429-36, 2008 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-19114185

RESUMO

OBJECTIVES: We sought to compare the usefulness of echocardiography and magnetic resonance imaging in neonates with a borderline small left ventricle. METHODS: The preoperative magnetic resonance and echocardiography studies of 20 consecutive patients (mean age 10 +/- 9 days) undergoing magnetic resonance imaging were analyzed. The diagnoses were aortic stenosis (n = 3), hypoplastic left heart complex (n = 12), and unbalanced atrioventricular septal defect (n = 5). The magnetic resonance imaging protocol included ventricular volumetry, flow measurements, and angiography. Potential left ventricular volumes, assuming an ideal geometric shape, were calculated by mathematically "unfolding" the compressed left ventricle. RESULTS: Left ventricular end-diastolic volume was 16.0 +/- 7.0 mL/m(2) of body surface area by echocardiography and 33.5 +/- 15.5 mL/m(2) by magnetic resonance imaging. Echocardiography consistently underestimated left ventricular volume and did not correlate with magnetic resonance. Of all echocardiographic parameters, mitral valve z-score was the best predictor of left ventricular end-diastolic volume by magnetic resonance (r = 0.77; P = .02). The average potential volume increase was 8.8% for aortic stenosis, 35.0% for atrioventricular septal defect and 23.0% for hypoplastic left heart complex patients. Aortic valve diameter did not correlate with flow volume in the ascending aorta. Sixteen (80%) of 20 patients underwent biventricular repair, without early mortality. Of these, only 5 (31.3%) had a preoperative left ventricular end-diastolic volume of more than 20 mL/m(2) by echocardiography. CONCLUSIONS: Magnetic resonance imaging is feasible in neonates with borderline left ventricular hypoplasia. Echocardiography does not accurately measure left ventricular hypoplasia in these patients and may unfairly preclude some patients from a biventricular repair in whom magnetic resonance is reassuring.


Assuntos
Ecocardiografia , Ventrículos do Coração/anormalidades , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Imageamento por Ressonância Magnética , Disfunção Ventricular Esquerda/diagnóstico , Tomada de Decisões , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Lactente , Recém-Nascido , Cuidados Pré-Operatórios , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda
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