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1.
Artigo em Inglês | MEDLINE | ID: mdl-38642340

RESUMO

OBJECTIVES: Predicting whether balloon atrial septostomy (BAS) will be necessary after birth for fetuses with d-transposition of the great arteries (d-TGA) remains challenging. We sought to determine whether measurements obtained during fetal maternal hyperoxygenation (MH) testing can improve our ability to predict need for postnatal BAS. METHODS: Forty-one mothers carrying fetuses with d-TGA with either intact ventricular septum or small ventricular septal defect measuring <3mm underwent MH testing between 33-38 weeks gestation. Patent foramen ovale (PFO) size, measured by 2D and color Doppler, patent ductus arteriosus (PDA) shunting (all antegrade versus bidirectional) was assessed in room air (RA) and during MH, blinded to postnatal outcome. BAS status and timing were recorded. RESULTS: Postnatally, 23 neonates underwent BAS while 18 did not, and 14 subjects underwent emergent BAS within 3 hours of life. By univariate analysis, PFO size measured both in RA and MH and all antegrade shunting in the PDA during MH predicted BAS. During MH testing, median PFO size by 2D measured 2.5mm (interquartile range, IQR, 2-3mm) in fetuses who underwent emergent BAS versus 4.1mm (IQR 3.4-5mm) in fetuses who did not undergo BAS (p<0.001). By cutpoint analysis, PFO size during MH testing ≤ 3.2mm predicted need for emergent BAS with sensitivity 93% and specificity 78%. CONCLUSIONS: In d-TGA, measurement of PFO size and direction of PDA shunting during MH testing improves our ability to predict need for BAS postnatally, although additional study is needed. We propose incorporating third trimester MH testing when planning deliveries of d-TGA fetuses. This article is protected by copyright. All rights reserved.

2.
Ultrasound Obstet Gynecol ; 59(5): 682-686, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-34494326

RESUMO

The mitral-aortic intervalvular fibrosa (MAIVF) is an avascular, fibrous structure that provides continuity between the anterior leaflet of the mitral valve and the aortic valve. Pseudoaneurysm of the MAIVF is rare and has been most commonly described in adults and, more rarely, in children following cardiac surgery or endocarditis. Few reports have been published on cases with congenital pseudoaneurysm of the MAIVF. Here, we describe five cases of congenital pseudoaneurysm of the MAIVF identified prenatally and an additional six cases diagnosed postnatally. This is an unusual finding of varying clinical significance, which can be isolated or associated with complex congenital heart disease but, importantly, can be identified and monitored in the fetus. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.


Assuntos
Falso Aneurisma , Procedimentos Cirúrgicos Cardíacos , Endocardite , Adulto , Falso Aneurisma/diagnóstico por imagem , Valva Aórtica/diagnóstico por imagem , Criança , Endocardite/patologia , Fibrose , Humanos , Valva Mitral/diagnóstico por imagem
3.
Clin Radiol ; 77(10): e776-e782, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35940927

RESUMO

AIM: To compare targeted and global liver stiffness measured by magnetic resonance elastography (MRE) with liver biopsy in patients who have undergone the Fontan procedure, and to assess the relationship between liver stiffness and fibrosis stage. MATERIALS AND METHODS: Targeted and global liver stiffness was compared with a quantification of liver fibrosis measured by percentage of Sirius Red (%SR) staining of biopsy samples. MRE values were compared with three other biopsy-scoring methods: Ishak, Scheuer/Ludwig-Batts/Metavir, and congestive hepatic fibrosis score (CHFS). Additionally, in patients who had two or more MRE studies, global liver stiffness was compared for longitudinal assessment. RESULTS: Thirty-four patients were included in the study, with a mean age of 16.2 years. There was no statistically significant correlation between MRE-derived liver stiffness and Ishak score, Metavir score, %SR staining, and CHFS score. Twenty patients had multiple MRE studies, with a mean age of 16.5 years, and these showed a statistically significant increase in mean liver stiffness from 3.72 to 4.68 (26% increase) within an average period of 24 months. CONCLUSIONS: The lack of correlation of liver stiffness with fibrosis stage observed in this study indicates that the effects of venous congestion in Fontan patients can confound the use of liver stiffness as a biomarker for fibrosis as assessed by percentage of SR staining, Ishak score, Metavir score, and CHFS score. These results provide motivation for further development of magnetic resonance imaging-based biomarkers to increase the specificity in the assessment of Fontan-associated liver disease. A steady increase in liver stiffness observed in these patients may be useful for longitudinal follow-up of liver health.


Assuntos
Técnicas de Imagem por Elasticidade , Técnica de Fontan , Hiperemia , Hepatopatias , Adolescente , Técnicas de Imagem por Elasticidade/métodos , Fibrose , Técnica de Fontan/efeitos adversos , Humanos , Hiperemia/diagnóstico por imagem , Hiperemia/etiologia , Hiperemia/patologia , Fígado/diagnóstico por imagem , Fígado/patologia , Cirrose Hepática/patologia , Hepatopatias/etiologia , Imageamento por Ressonância Magnética/métodos , Estudos Retrospectivos
4.
Ultrasound Obstet Gynecol ; 55(4): 516-522, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-30989734

RESUMO

OBJECTIVE: EXTrauterine Environment for Neonatal Development (EXTEND) is a system to support ongoing fetal growth and organ development in an extrauterine environment, utilizing a pumpless low-resistance oxygenator circuit. The aim of this study was to evaluate hemodynamics and cardiac function in fetal sheep sustained on the EXTEND system. METHODS: This was a prospective study of fetal sheep supported for a minimum of 3 weeks on EXTEND. Hemodynamic parameters were assessed weekly and included heart rate, mean arterial pressure (MAP), Doppler-echocardiography-derived cardiac output (CO), pulsatility indices (PIs) of the fetal middle cerebral artery (MCA), umbilical artery (UA) and ductus venosus and cardiac function, as assessed by speckle-tracking-derived global longitudinal strain and strain rate in the right (RV) and left (LV) ventricles. Parameters were compared at 0 days and 1, 2 and 3 weeks following placement on EXTEND. RESULTS: Of 10 fetal sheep enrolled, seven survived for 3 weeks and were included in the analysis. Median gestational age at cannulation was 107 (range, 95-109) days. Heart rate decreased and MAP increased significantly, but within acceptable ranges, during the study period. The quantities and relative ratios of right and left CO remained stable within the anticipated physiological range throughout the study period. Vascular tracings and PIs appeared to be similar to those seen normally in the natural in-utero state, with MCA-PI being higher than UA-PI. UA tracings demonstrated maintained abundant diastolic flow despite the absence of placental circulation. In both the RV and LV, strain decreased significantly at 1 and 2 weeks relative to baseline but returned to baseline values by week 3. CONCLUSIONS: The EXTEND mechanical support system replicates natural physiology and creates a stable and sustainable cardiovascular construct that supports growth over a 3-week period. However, there is a period of depressed contractility within the first week with subsequent improvement by week 3. This may reflect a period of physiological accommodation that warrants further investigation. This study lays the foundation for further exploration as the EXTEND system moves towards human application. © 2019 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.


Assuntos
Cardiotocografia/métodos , Ecocardiografia Doppler/métodos , Oxigenação por Membrana Extracorpórea , Coração Fetal/diagnóstico por imagem , Feto/diagnóstico por imagem , Animais , Animais Recém-Nascidos/embriologia , Animais Recém-Nascidos/crescimento & desenvolvimento , Débito Cardíaco , Feminino , Desenvolvimento Fetal/fisiologia , Coração Fetal/embriologia , Coração Fetal/crescimento & desenvolvimento , Feto/embriologia , Feto/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Hemodinâmica , Artéria Cerebral Média/embriologia , Gravidez , Estudos Prospectivos , Fluxo Pulsátil , Ovinos , Fatores de Tempo , Artérias Umbilicais/embriologia
5.
Ultrasound Obstet Gynecol ; 52(4): 473-478, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28976608

RESUMO

OBJECTIVES: Compared with normal fetuses, fetuses with hypoplastic left heart syndrome (HLHS) have smaller brain volumes and are at higher risk of brain injury, possibly due to diminished cerebral blood flow and oxygen content. By increasing cerebral oxygen delivery, maternal hyperoxygenation (MH) might improve brain development and reduce the risk of brain injury in these fetuses. This study investigated whether gestational age and baseline cerebrovascular resistance affect the response to MH in fetuses with HLHS. METHODS: The study population comprised 43 fetuses with HLHS or HLHS variant referred for fetal echocardiography between January 2004 and September 2008. Middle cerebral artery (MCA) pulsatility index (PI), a surrogate measure of cerebrovascular resistance, was assessed between 20 and 41 weeks' gestation at baseline in room air (RA) and after 10 min of MH. Z-scores of MCA-PI were generated. A mixed-effects model was used to determine whether change in MCA-PI depends upon gestational age and baseline MCA-PI. RESULTS: In RA and following MH, MCA-PI demonstrated a curvilinear relationship with gestational age in fetuses with HLHS, peaking at around 28 weeks and then falling more steeply near term. MCA-PI Z-score declined in a linear manner, such that it was 1.4 SD below that in normal fetuses at 38 weeks. Increase in MCA-PI Z-score after MH was first seen at ≥ 28 weeks. A baseline MCA-PI Z-score ≤ -0.96 was predictive of an increase in cerebrovascular resistance in response to MH. CONCLUSION: In fetuses with HLHS, MCA-PI first increases in response to MH at ≥ 28 weeks' gestation. A baseline MCA-PI Z-score ≤ -0.96 predicts an increase in cerebrovascular resistance in response to MH. These results may have implications for clinical trials utilizing MH as a neuroprotective agent. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.


Assuntos
Circulação Cerebrovascular/fisiologia , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Artéria Cerebral Média/fisiopatologia , Transtornos do Neurodesenvolvimento/prevenção & controle , Oxigenoterapia , Oxigênio/sangue , Placenta/irrigação sanguínea , Adulto , Ecocardiografia , Feminino , Monitorização Fetal , Idade Gestacional , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/embriologia , Síndrome do Coração Esquerdo Hipoplásico/terapia , Recém-Nascido , Artéria Cerebral Média/diagnóstico por imagem , Artéria Cerebral Média/embriologia , Mães , Transtornos do Neurodesenvolvimento/fisiopatologia , Transtornos do Neurodesenvolvimento/terapia , Placenta/metabolismo , Gravidez , Fluxo Pulsátil , Resultado do Tratamento , Ultrassonografia Pré-Natal
9.
Ultrasound Obstet Gynecol ; 45(6): 664-9, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25296951

RESUMO

OBJECTIVES: Aneurysm of the atrial septum (AAS) with excessive excursion of septum primum into the left atrium is an uncommon and relatively benign fetal condition associated with impediment to left ventricular (LV) filling and the appearance of a slender, but apex-forming, LV on fetal echocardiography. Impediment to filling can be severe, creating the image of LV hypoplasia with retrograde aortic flow. We hypothesize that maternal hyperoxygenation alters atrial septal position, improves LV filling, and normalizes aortic flow in fetuses with AAS by increasing fetal pulmonary venous return. METHODS: Fetal echocardiography was performed prior to, and at 10 min of, maternal hyperoxygenation in 12 fetuses with AAS who were referred to our center because of LV hypoplasia. Atrial septal excursion (ASE), LV and right ventricular (RV) sphericity index (SI) and direction of flow in the aortic isthmus, as determined by Doppler, were measured. RESULTS: With maternal hyperoxygenation, mean ± SD ASE decreased (0.76 ± 0.17 before maternal hyperoxygenation vs 0.53 ± 0.23 after maternal hyperoxygenation; P < 0.01), consistent with increased pulmonary venous return, LV-SI increased (0.29 ± 0.06 vs 0.42 ± 0.06; P < 0.001), indicating increased LV filling, and the direction of aortic isthmus flow changed from retrograde in all cases prior to maternal hyperoxygenation to antegrade in 10 and to bidirectional in two. RV-SI remained unchanged (0.53 ± 0.13 vs 0.52 ± 0.10; P = 0.7). CONCLUSIONS: In cases of AAS, short-term maternal hyperoxygenation increases fetal pulmonary venous return, substantially alters LV geometry and promotes antegrade flow in the aortic isthmus. This demonstrates proof-of-concept that maternal hyperoxygenation can improve filling of the left side of the fetal heart in AAS.


Assuntos
Coração Fetal/fisiopatologia , Aneurisma Cardíaco/terapia , Ventrículos do Coração/anormalidades , Oxigenoterapia/métodos , Ecocardiografia Doppler em Cores , Feminino , Coração Fetal/diagnóstico por imagem , Idade Gestacional , Aneurisma Cardíaco/diagnóstico por imagem , Aneurisma Cardíaco/embriologia , Átrios do Coração , Humanos , Gravidez , Estudos Retrospectivos , Ultrassonografia Pré-Natal/métodos
10.
Ultrasound Obstet Gynecol ; 43(3): 297-302, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23554091

RESUMO

OBJECTIVES: Fetuses with pulmonary outflow tract obstruction (POTO) have altered blood flow to the pulmonary vasculature. We sought to determine whether pulmonary vascular impedance, as assessed by the pulsatility index (PI), is different in fetuses with POTO compared with normal controls. METHODS: Branch pulmonary artery PI was evaluated in age-matched normal control fetuses (n=22) and 20 POTO fetuses (pulmonary stenosis n=15, pulmonary atresia n=5). Pulsed-wave Doppler was performed in the proximal (PA1), mid (PA2) and distal (PA3) branch pulmonary artery. The direction of flow in the ductus arteriosus was noted. The study and control groups were compared with Student's t-test and ANOVA. A linear mixed model evaluated the relationship between PI and ductus arteriosus flow patterns. RESULTS: There was no difference in PI between control, pulmonary stenosis and pulmonary atresia subjects at PA1 and PA2; however, there was a significant difference at PA3. Subjects with pulmonary atresia had a lower PI at PA3 than did controls (P=0.003) and pulmonary stenosis subjects (P=0.003). Subjects with retrograde flow in the ductus arteriosus had lower PIs in PA2 and PA3 than did those with antegrade flow (P=0.01 and 0.005, respectively). The PI in PA3 was lower in fetuses that required prostaglandin postnatally than in those that did not (P=0.008). CONCLUSIONS: Fetuses with pulmonary atresia or severe pulmonary stenosis with retrograde flow in the ductus arteriosus have decreased PI in the distal pulmonary vasculature. Our findings indicate the capacity of the fetal pulmonary vasculature to vasodilate in response to anatomical obstruction of flow.


Assuntos
Alprostadil/administração & dosagem , Canal Arterial/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Atresia Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/diagnóstico por imagem , Vasodilatadores/administração & dosagem , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo/efeitos dos fármacos , Ecocardiografia Doppler de Pulso , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Gravidez , Artéria Pulmonar/anormalidades , Artéria Pulmonar/embriologia , Atresia Pulmonar/tratamento farmacológico , Estenose da Valva Pulmonar/tratamento farmacológico , Estudos Retrospectivos , Sensibilidade e Especificidade , Ultrassonografia Pré-Natal , Obstrução do Fluxo Ventricular Externo/tratamento farmacológico , Obstrução do Fluxo Ventricular Externo/embriologia
11.
Ultrasound Obstet Gynecol ; 41(1): 47-53, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-22605656

RESUMO

OBJECTIVE: To identify prenatal echocardiographic markers that could predict the need for neonatal intervention in fetuses with right ventricular outflow tract obstruction. METHODS: This was a retrospective study of 52 fetuses with right ventricular outflow tract obstruction. Echocardiograms were evaluated for fetuses with either two-ventricle anatomy with a large ventricular septal defect or single-ventricle anatomy. Fetuses with pulmonary atresia were excluded. Parameters were compared between groups that did and did not require an intervention at age < 30 days. RESULTS: Fifty-two fetuses were studied; 20 (38%) underwent neonatal intervention and 32 (62%) did not. The most common diagnosis was tetralogy of Fallot (n = 32). Fetuses with two ventricles that required an intervention had lower pulmonary valve diameter Z-score (PV-Z-score) (-4.8 ± 2.1 vs. -2.6 ± 1.1; P = 0.0002) and lower pulmonary valve to aortic valve annular diameter ratio (PV/AoV) (0.53 ± 0.15 vs. 0.66 ± 0.1; P = 0.003). Using a PV/AoV ratio of < 0.6 or a PV-Z-score of < -3 at final echocardiographic examination was highly sensitive (92%) but poorly specific (50%), whereas classifying direction of flow in the ductus arteriosus as either normal (all pulmonary-to-aorta) or abnormal (aorta-to-pulmonary or bidirectional) was both highly sensitive (100%) and specific (95%) for predicting the need for a neonatal intervention. Parameters for the single-ventricle cohort did not reach statistical significance. CONCLUSIONS: Analysis of the pulmonary outflow tract and ductus arteriosus flow in the fetus with complex congenital heart disease can aid in identifying those that will require a neonatal intervention to augment pulmonary blood flow. This has important implications for the planning of delivery strategies.


Assuntos
Ecocardiografia Doppler de Pulso/métodos , Ecocardiografia Doppler/métodos , Comunicação Interventricular/diagnóstico por imagem , Tetralogia de Fallot/diagnóstico por imagem , Ultrassonografia Pré-Natal/métodos , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Previsões , Idade Gestacional , Comunicação Interventricular/terapia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Recém-Nascido , Estudos Retrospectivos , Sensibilidade e Especificidade , Tetralogia de Fallot/terapia , Obstrução do Fluxo Ventricular Externo/terapia
12.
Ultrasound Obstet Gynecol ; 40(1): 62-7, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22407644

RESUMO

OBJECTIVE: We sought to determine whether the presence or absence of aortic obstruction impacts cerebrovascular resistance in fetuses with single-ventricle (SV) congenital heart disease (CHD). METHODS: Pulsatility indices (PIs) were recorded for the middle cerebral artery (MCA) and the umbilical artery (UA) from 18 to 40 weeks' gestation in 59 fetuses (163 examinations) with SV-CHD with unobstructed aortic flow, yet decreased pulmonary flow, in 72 fetuses (170 examinations) with obstructed aortic flow and hypoplastic left heart syndrome (HLHS) and in 92 normal fetuses (92 examinations). The cerebral-to-placental resistance (CPR) was calculated as the MCA-PI/UA-PI. Z-scores were generated for the MCA-PI and the UA-PI in order to make comparisons independent of gestational age. Statistical analyses were performed using one-way ANOVA with post-hoc testing. Trends in these variables over the course of gestation were assessed using linear regression and univariate ANOVA. RESULTS: The MCA-PI and the CPR were significantly lower in SV fetuses with aortic obstruction compared with SV fetuses with pulmonary obstruction and with normal fetuses. Moreover, the MCA-PI decreased significantly for SV fetuses with aortic obstruction over the course of gestation. In contrast, the MCA-PI was higher over the course of gestation in SV fetuses with pulmonary obstruction compared with normal fetuses. CONCLUSION: In fetuses with SV-CHD, cerebrovascular resistance varies substantially between fetuses with and without aortic obstruction. Compared with normal fetuses, cerebrovascular resistance is decreased in SV fetuses with aortic obstruction, yet increased in SV fetuses with pulmonary obstruction. In fetuses with SV physiology, inherent differences in cerebral blood flow may underlie postnatal neurodevelopmental outcomes.


Assuntos
Circulação Cerebrovascular , Deficiências do Desenvolvimento/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Artéria Cerebral Média/fisiopatologia , Fluxo Pulsátil , Artérias Umbilicais/fisiopatologia , Análise de Variância , Velocidade do Fluxo Sanguíneo , Deficiências do Desenvolvimento/diagnóstico por imagem , Deficiências do Desenvolvimento/etiologia , Feminino , Idade Gestacional , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/embriologia , Humanos , Lactente , Recém-Nascido , Masculino , Artéria Cerebral Média/diagnóstico por imagem , Artéria Cerebral Média/embriologia , Gravidez , Ultrassonografia Pré-Natal , Artérias Umbilicais/diagnóstico por imagem , Artérias Umbilicais/embriologia , Resistência Vascular
16.
Ultrasound Obstet Gynecol ; 36(6): 682-6, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-20503245

RESUMO

OBJECTIVE: Lower urinary tract obstruction (LUTO) with massive bladder distension impacts on a variety of developing organ systems with consequent morbidity and mortality. The impact of LUTO on the fetal cardiovascular system has not previously been investigated. We hypothesize that a giant, distended bladder within the pelvis may cause vascular compression with observable consequences. METHODS: Fetal echocardiography was performed in 42 fetuses with LUTO and compared with 35 normal controls matched for gestational age. Parameters investigated included cardiothoracic ratio, presence or absence of ventricular hypertrophy and pericardial effusion. Doppler echocardiographic examination of mitral and tricuspid valve inflow and the ductus venosus was performed. To assess arterial vascular impedance, pulsatility indices (PIs) were calculated for segments of the descending aorta and right and left iliac arteries (RIA and LIA). RESULTS: In the LUTO group an increased cardiothoracic ratio was seen in nine (21%), ventricular hypertrophy in 12 (29%) and small pericardial effusion in 15 (36%). Filling characteristics of the right ventricle in the LUTO group demonstrated greater dependency upon atrial contraction, and ductus venosus flow demonstrated higher downstream impedance to filling, than in controls, suggesting altered compliance. The LUTO group also had lower distal descending aorta PI (1.53 ± 0.38 vs. 1.76 ± 0.44, P = 0.04), lower RIA-PI (1.31 ± 0.29 vs. 1.76 ± 0.44, P < 0.001) and lower LIA-PI (1.41 ± 0.44 vs. 1.73 ± 0.31, P < 0.01) than controls, suggesting vascular compression and increased impedance to flow. CONCLUSIONS: LUTO with giant urinary bladder compresses the iliac arteries, which may lead to increased afterload. Further study is warranted to examine the impact of fetal treatment such as bladder drainage on these cardiovascular parameters.


Assuntos
Doenças Cardiovasculares/diagnóstico por imagem , Coração Fetal/diagnóstico por imagem , Obstrução Uretral/diagnóstico por imagem , Doenças da Bexiga Urinária/diagnóstico por imagem , Bexiga Urinária/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo/fisiologia , Doenças Cardiovasculares/embriologia , Doenças Cardiovasculares/etiologia , Ecocardiografia Doppler , Feminino , Coração Fetal/fisiopatologia , Idade Gestacional , Humanos , Gravidez , Ultrassonografia Pré-Natal , Obstrução Uretral/complicações , Obstrução Uretral/embriologia , Bexiga Urinária/irrigação sanguínea , Bexiga Urinária/embriologia , Doenças da Bexiga Urinária/embriologia , Doenças da Bexiga Urinária/fisiopatologia
17.
Ultrasound Obstet Gynecol ; 36(4): 465-70, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-20499409

RESUMO

OBJECTIVES: To review our experience with the prenatal diagnosis of hypoplastic left heart syndrome (HLHS). Our goal was to establish the benchmark for perinatal and early surgical outcome in the current era, from a center with an aggressive surgical approach and a cohort with a high level of intention-to-treat. METHODS: Outcome was assessed in fetuses with HLHS following stratification into high-risk and standard-risk categories. High risk was defined as the presence of any of the following: extracardiac, genetic or chromosomal anomalies; prematurity of < 34 weeks' gestation; additional cardiac findings such as intact or highly restrictive atrial septum, severe degree of tricuspid regurgitation or severe ventricular dysfunction. Standard risk was defined as absence of these risk factors. RESULTS: Of 240 fetuses evaluated over 5 years, 162 (67.5%) were in the standard-risk group and 78 (32.5%) were in the high-risk group. Of the 240 sets of parents, 38 (15.8%) chose termination or non-intervention at birth at initial prenatal counseling and 185 of the neonates (77.1%) underwent first-stage Norwood surgery with 155 surviving and 30 deaths, giving an overall Norwood operative survival of 83.8%. Breakdown by risk class reveals a significant Norwood operative survival advantage for the standard-risk group (92.8%) over the high-risk group (56.5%) (P < 0.001). CONCLUSIONS: Following prenatal diagnosis of HLHS, families should be strongly encouraged to undertake comprehensive prenatal evaluation in order to obtain an accurate prognosis. One-third have additional risk factors that limit survival outcome, however two-thirds do not and have an excellent chance of early survival.


Assuntos
Benchmarking , Procedimentos Cirúrgicos Cardíacos/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Procedimentos Cirúrgicos Cardíacos/normas , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Gravidez , Resultado da Gravidez , Diagnóstico Pré-Natal , Prognóstico , Fatores de Risco , Análise de Sobrevida , Ultrassonografia
18.
Fetal Diagn Ther ; 27(2): 101-5, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20090295

RESUMO

Congenital tricuspid valve disease (Ebstein's anomaly, tricuspid valve dysplasia) with severe tricuspid regurgitation and cardiomegaly is associated with poor prognosis. Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease. Fetal lung volumetric assessments have been used in an attempt to predict viability of fetuses using ultrasonogram and prenatal MRI. We describe a fetus with tricuspid dysplasia, severe tricuspid regurgitation, right atrial enlargement and markedly reduced lung volumes. The early gestational onset of cardiomegaly with bilateral lung compression raised the possibility of severe lung hypoplasia with decreased broncho-alveolar development. Use of fetal echocardiography with measurement of pulmonary artery size combined with prenatal MRI scanning of lung volumes resulted in an improved understanding of this anomaly and directed the management strategy towards a successful Fontan circulation.


Assuntos
Anomalia de Ebstein/diagnóstico , Pulmão/embriologia , Diagnóstico Pré-Natal/métodos , Artéria Pulmonar/embriologia , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/anormalidades , Adulto , Cardiomegalia/diagnóstico por imagem , Cardiomegalia/embriologia , Cardiomegalia/cirurgia , Ponte Cardiopulmonar , Anomalia de Ebstein/cirurgia , Feminino , Doenças Fetais/diagnóstico por imagem , Idade Gestacional , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , Humanos , Recém-Nascido , Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética , Gravidez , Artéria Pulmonar/cirurgia , Valva Tricúspide/embriologia , Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/cirurgia , Ultrassonografia Pré-Natal
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