Pregnancy outcome of fetuses with a diagnosis of hypoplastic left ventricle on prenatal sonography. (57/199)

OBJECTIVE: The goal of our study was to assess the pregnancy outcome of fetuses with a diagnosis of hypoplastic left heart syndrome (HLHS) on prenatal sonography to determine the frequency of intrauterine fetal demise (IUFD) and any factors associated with IUFD. METHODS: We reviewed all cases with the diagnosis of HLHS on prenatal sonography at our institution from 1992 through 2003. Data collected included gestational age at diagnosis, sonographic findings, karyotype testing, and pregnancy outcome. RESULTS: Our study included 176 fetuses with HLHS. One hundred thirty-four fetuses were liveborn; 32 pregnancies were terminated; 3 IUFDs occurred; and outcome was unknown in 7. Of the 134 liveborn fetuses, 2 had abnormal karyotypes and 30 had other anomalies. Two of the 3 fetuses with IUFD had abnormal karyotypes, 1 with trisomy 13 and 1 with trisomy 18, and both had other anomalies on sonography. The third fetus with IUFD had no other anomalies but was 1 of triplets, and the karyotype was unknown. CONCLUSIONS: Fetuses with HLHS diagnosed prenatally who have normal chromosomes are unlikely to die in utero.  (+info)

Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. (58/199)

BACKGROUND: After the Norwood operation, a patient's suitability for proceeding to a bidirectional cavopulmonary connection (BCPC) is assessed by a combination of echocardiography and diagnostic cardiac catheterization. In this study, we describe the results of 37 patients who underwent cardiovascular magnetic resonance (MR) assessment before BCPC. METHODS AND RESULTS: Cardiovascular MR and echocardiography were performed in 37 infants with hypoplastic left heart syndrome before BCPC, and the findings were compared with surgical findings. MR assessment of ventricular function and valvar regurgitation were compared with echocardiography. MR exhibited high sensitivity and specificity for identification of neoaortic (sensitivity 86%, specificity 97%) and left pulmonary artery (sensitivity 100%, specificity 94%) obstruction. Echocardiography exhibited poor sensitivity for identification of vascular stenosis. The mean right ventricular ejection fraction calculated from the MR data was 50+/-10%. There was general agreement between MR and echocardiographic measures of ventricular function, although patients with good function on echocardiography demonstrated a wide range of ejection fractions. There was good agreement between MR and echocardiography for identification of valvar regurgitation. CONCLUSIONS: Cardiovascular MR can be used to define ventricular and valvar function and vascular anatomy in infants with hypoplastic left heart syndrome after the Norwood operation. We have shown how this information can be used to plan the BCPC and identify any revisions or additional valvar surgery.  (+info)

Investigation of intensity thresholds for ultrasound tissue erosion. (59/199)

Our previous studies have shown that short intense pulses delivered at certain pulse repetition frequencies (PRF) can achieve localized, clean erosion in soft tissue. In this paper, the intensity thresholds for ultrasound induced erosion and the effects of pulse intensity on erosion characterized by axial erosion rate, perforation area and volume erosion rate were investigated on in vitro porcine atrial wall tissue. Ultrasound pulses with a 3-cycle pulse duration and a 20-kHz PRF were delivered by a 788-kHz single element focused transducer. I(SPPA) values of 1000 to 9000 W/cm2 were tested. Results show the following: (1) the estimated intensity threshold for generating erosion was at I(SPPA) of 3220 W/cm2; (2) the axial erosion rate increased with higher intensity at I(SPPA) < or = 5000 W/cm2, while decreased with higher intensity at I(SPPA) > or = 5000 W/cm2; and (3) the perforation area and the volume erosion rate increased with higher intensity.  (+info)

Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome. (60/199)

OBJECTIVE: Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age. METHODS: A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 21 (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 +/- 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration. RESULTS: The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 +/- 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores. CONCLUSIONS: Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age.  (+info)

Prenatal diagnosis of ventriculocoronary arterial communication in fetuses with hypoplastic left heart syndrome. (61/199)

OBJECTIVE: The purpose of this series was to describe the fetal echocardiographic findings in hypoplastic left heart syndrome with aortic atresia and ventriculocoronary arterial communication and implications of these findings. METHODS: We describe 2 fetuses with hypoplastic left heart syndrome with ventriculocoronary arterial communication diagnosed at 29 and 20 weeks' gestation, respectively. The underlying cardiac anatomy consisted of a hypoplastic left heart and mitral stenosis with aortic atresia. We used color Doppler and pulsed Doppler sonography on the surface of the myocardium to specifically look for coronary arterial flow. RESULTS: By color Doppler sonography, ventriculocoronary arterial communication was shown between the left ventricular cavity and the left coronary artery with characteristic bidirectional flow on pulsed Doppler examination. There was no mitral regurgitation. The left ventricular myocardium was substantially hypertrophied. The first patient underwent surgical Norwood palliation and died after a prolonged postoperative course. The second patient underwent stenting of the arterial duct and bilateral pulmonary artery banding in the catheterization laboratory but died after a few weeks. Implications of ventriculocoronary arterial communication in association with hypoplastic left heart syndrome are discussed. CONCLUSIONS: It is possible to accurately diagnose ventriculocoronary arterial communication on fetal echocardiography. The presence of ventriculocoronary arterial communication is seen exclusively in a subgroup of patients with an aortic atresia and mitral stenosis variant of hypoplastic left heart syndrome. The prognosis is poor in this subgroup of patients.  (+info)

Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome: patient selection for fetal intervention. (62/199)

BACKGROUND: Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for fetal intervention. The aim of this study was to define echocardiographic features associated with progression of midgestation fetal AS to HLHS. METHODS AND RESULTS: Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at < or =30 weeks' gestation. Of 23 live-born patients with available follow-up data, 17 had HLHS and 6 had a biventricular circulation. At the time of diagnosis, LV length, mitral valve, aortic valve, and ascending aortic diameter Z-scores did not differ between fetuses that ultimately developed HLHS and those that maintained a biventricular circulation postnatally. However, all of the fetuses that progressed to HLHS had retrograde flow in the transverse aortic arch (TAA), 88% had left-to-right flow across the foramen ovale, 91% had monophasic mitral inflow, and 94% had significant LV dysfunction. In contrast, all 6 fetuses with a biventricular circulation postnatally had antegrade flow in the TAA, biphasic mitral inflow, and normal LV function. With advancing gestation, growth arrest of left heart structures became evident in fetuses developing HLHS. CONCLUSIONS: In midgestation fetuses with AS and normal LV length, reversed flow in the TAA and foramen ovale, monophasic mitral inflow, and LV dysfunction are predictive of progression to HLHS. These physiological features may help refine patient selection for fetal intervention to prevent the progression of AS to HLHS.  (+info)

In-hospital mortality for children with hypoplastic left heart syndrome after stage I surgical palliation: teaching versus nonteaching hospitals. (63/199)

OBJECTIVES: Teaching hospitals are perceived to provide a higher quality of care for the treatment of rare disease and complex patients. A substantial proportion of stage I palliation for hypoplastic left heart syndrome (HLHS) may be performed in nonteaching hospitals. This study compares the in-hospital mortality of stage I palliation between teaching and nonteaching hospitals. METHODS: The authors conducted a retrospective cohort study using the Kids' Inpatient Database 1997 and 2000. Patients with HLHS undergoing stage I palliation were identified using International Classification of Diseases, Ninth Revision, Clinical Modification diagnostic and procedural codes. RESULTS: Seven hundred fifty-four and 880 discharges of children with HLHS undergoing stage I palliation in 1997 and 2000, respectively, were identified. The in-hospital mortality for the study population was 28% in 1997 and 24% in 2000. Twenty percent of stage I palliation operations were performed in nonteaching hospitals in 1997. Two percent of operations were performed in nonteaching hospitals in 2000. In 1997 only, in-hospital mortality remained higher in nonteaching hospitals after controlling for stage I palliation hospital volume and condition-severity diagnoses. Low-volume hospitals performing stage I palliation were associated with increased in-hospital mortality in 1997 and 2000. CONCLUSIONS: Patients with HLHS undergoing stage I palliation in nonteaching hospitals experienced increased in-hospital mortality in 1997. A significant reduction in the number of stage I palliation procedures performed in nonteaching hospitals occurred between 1997 and 2000. This centralization of stage I palliation into teaching hospitals, along with advances in postoperative medical and surgical care for these children, was associated with a decrease in mortality. Patients in low-volume hospitals performing stage I palliation continued to experience increased mortality in 2000.  (+info)

Factors affecting technical success of fetal aortic valve dilation. (64/199)

OBJECTIVE: We have reported previously that valve dilation enhances growth of cardiac structures and may prevent hypoplastic left heart syndrome (HLHS) in fetuses with critical aortic stenosis. We aimed to investigate maternal/fetal factors which may affect the technical success of fetal valvuloplasty, and to describe perinatal complications of the procedure. METHODS: This was a descriptive series of 22 fetuses diagnosed with critical aortic stenosis developing into HLHS which underwent intervention by valvuloplasty. Initially this was attempted using a percutaneous approach; reassessment after our first five attempts, only one of which was successful, led to the introduction of the option of laparotomy. Technical success was defined as balloon inflation across the aortic annulus and a broader jet through the aortic valve as assessed by Doppler. Data collected included body mass index, demographic variables, ultrasound findings and postprocedure interventions. RESULTS: Technical success increased significantly if maternal laparotomy was an option (83.3% vs. 20.0%, P = 0.017). Laparotomy was performed in 66.6% (12/18) of cases. There was a learning curve that showed an increase in success rate and decrease in need for laparotomy over the 3-year study period. Neither the need for laparotomy nor the chances of technical success were predictable by gestational age, body mass index or placental location. Tocolysis was limited to perioperative prophylaxis; one woman experienced wound infection and fluid overload. Postoperatively, three fetuses died and two delivered prematurely, 2 and 7 weeks after intervention. CONCLUSION: Fetal aortic valvuloplasty can be performed with technical success, with low fetal loss rate and few maternal complications. While the need for laparotomy cannot be predicted, having it available as an option improves the technical success rate.  (+info)