Assessment of fetal cardiomyopathy in early-stage twin-twin transfusion syndrome: comparison between commonly reported cardiovascular assessment scores.

OBJECTIVES: To assess the relationship between commonly reported fetal cardiomyopathy scoring systems in early-stage twin-twin transfusion syndrome (TTTS). METHODS: We reviewed retrospectively 100 cases of Quintero Stages I and II TTTS referred to our center for evaluation from 2008 to 2010. The cases were divided into groups of 25, representing each of four grades of TTTS cardiomyopathy as assessed by Cincinnati stage: no cardiomyopathy, Stage IIIa, Stage IIIb and Stage IIIc. Spearman correlation (rs ) was calculated between the Children's Hospital of Philadelphia (CHOP) score, cardiovascular profile score (CVPS), Cincinnati stage and myocardial performance index (MPI). RESULTS: There was a weak correlation between the Cincinnati stage and the CHOP score (rs = 0.36) and CVPS (rs = -0.39), while correlation was strong between the CHOP score and CVPS (rs = -0.72). MPI elevation was concordant with Cincinnati stage more frequently (82% of cases) than were ventricular hypertrophy (43%) or atrioventricular valve regurgitation (28%). 51% of fetuses with minimally elevated CHOP score (0-1) and 48% of fetuses with minimally depressed CVPS (9-10) had significant elevation (Z-score ≥ +3) in right ventricular or left ventricular MPI. CONCLUSIONS: MPI has a strong influence on grading the severity of fetal cardiomyopathy using the Cincinnati stage among fetuses with mild TTTS. Furthermore, significant elevation of the MPI is common among fetuses with mild disease as assessed by the CHOP score and CVPS. These differences should be understood when assessing and grading cardiomyopathy in TTTS, particularly in early (Quintero Stages I and II) disease.

Twin-reversed arterial perfusion sequence: pre- and postoperative cardiovascular findings in the 'pump' twin.

OBJECTIVES: To assess cardiovascular findings in twin-reversed arterial perfusion (TRAP) sequence pre- and post-therapy and compare these findings to traditional obstetric markers, defined as acardius to pump twin weight ratio and presence of polyhydramnios. METHODS: This was a retrospective review of 27 cases of TRAP sequence diagnosed between 2004 and 2008. Echocardiographic data included indexed cardiac output and functional and anatomic parameters. Ultrasound reports were reviewed for acardius to pump twin weight ratio and polyhydramnios. We assessed the relationship between cardiac output and the remaining cardiac/obstetric variables obtained pre- and post-treatment. RESULTS: Twenty-three subjects had complete echocardiographic data sets at initial evaluation (mean gestational age, 20.4 +/- 2.5 weeks) and, of these, post-treatment echocardiographic evaluation was available in 10. Six of seven (86%) pump twins with elevated indexed cardiac output had significant cardiovascular compromise. Most fetuses with abnormal cardiac output or right ventricular dysfunction normalized post-therapy. There was no relationship between cardiac output and obstetric markers. CONCLUSIONS: Elevated indexed cardiac output is strongly associated with cardiovascular compromise. Traditional obstetric prognosticators do not correlate with cardiovascular derangements. In pump twins with cardiac compromise, postoperative cardiovascular status improves acutely. Given this analysis, we conclude that assessment of cardiovascular findings should be incorporated into the management and treatment of TRAP sequence.

Accuracy of fetal echocardiography: a cardiac segment-specific analysis.

OBJECTIVE: In patients with congenital heart disease, comprehensive, segment-specific analysis of cardiac anatomy has become 'the standard of care', largely as a result of improvements in cardiac imaging technology. Our aim was to apply segment-specific standards to assess the accuracy of fetal echocardiography. METHODS: This was a retrospective review of all fetal echocardiograms (n = 915) performed at our center between August 1998 and June 2003. Of these, 100 studies had congenital heart disease findings and corresponding postnatal studies on the same patients for comparison. An expert independent pediatric echocardiologist, using the standards of accuracy expected of postnatal echocardiography, assessed the studies for the following cardiac segments: abdominal situs, systemic venous return (VR), pulmonary VR, atria, atrioventricular valves, ventricular septum, ventricular hypoplasia, ventricular morphology, semilunar valves, great arterial relation and aortic arch. Sensitivity, specificity, and positive and negative predictive values were calculated for each segment. RESULTS: Specificity and negative predictive value were high for all cardiac segments (range, 82-100%). Sensitivity and positive predictive value were similarly high (range, 83-100%) for most cardiac segments, but were only 50-88% for systemic VR, pulmonary VR and aortic arch segments. CONCLUSIONS: Fetal echocardiography has excellent diagnostic accuracy in describing intracardiac anatomy. However, despite both technological advances and improved physician awareness, assessment of systemic VR, pulmonary VR, and aortic arch anatomy remain challenging.

Noncircumferential myofiber function: impact on early diastolic filling in children.

Circumferential and noncircumferential myofiber contraction may have varying impact on systolic and diastolic function. The purpose of this study was to determine the relation of circumferential, longitudinal, and oblique fiber shortening to early diastolic filling in children. Twenty-five patients (8.1 +/- 5.6 years of age; 12 boys and 13 girls) with normal echocardiograms and no heart disease had prospective echocardiographic evaluation of circumferential (shortening fraction, fractional area change), longitudinal (left ventricular axial shortening), combined circumferential and longitudinal (left ventricular ejection fraction), oblique (left ventricular systolic twist [LVST]) shortening, and early diastolic filling. Mean LVST was 16 +/- 8 degrees. There was no relation between early diastolic filling indexes and indexes of circumferential or longitudinal shortening. However, there was a significant inverse relation between heart rate-corrected E-wave acceleration time and LVST (r = 0.63, P <.001). Oblique fiber shortening affects early diastolic filling in children. Describing the functional role of noncircumferential left ventricular myofibers may improve our understanding of global left ventricular function.

Unique echocardiographic features associated with deployment of the Amplatzer atrial septal defect device.

The purpose of this study was to describe the unique echocardiographic findings associated with deployment of the Amplatzer atrial septal defect (ASD) device. Thirty-five patients (2 to 40 years old; 23 female and 12 male patients) underwent echocardiography during attempted ASD closure with the Amplatzer device. Transesophageal and transthoracic echocardiograms were performed during the placement and follow-up of the device, respectively. In 5 patients, the device was not deployed because of transesophageal echocardiography (TEE) findings (an exceedingly large defect in 3 patients, partial obstruction of the upper right pulmonary vein by the device in 1, and complex atrial septal anatomy in 1). In the remaining 30 patients, after deployment but before release, the device distorted the atrial septum from the normal vertical orientation to an oblique transverse orientation. Excessive septal distortion (i.e., > or =90 degrees in 1 patient) was associated with device embolization upon release. In other patients, TEE also identified mild splaying of the device on the aortic wall, mild abutment of the device upon the mitral valve, and temporary partial obstruction of pulmonary vein flow. Color Doppler revealed residual shunts in 21 of 29 patients immediately after release, but in none of 15 patients at 1-year follow-up. Transesophageal echocardiography is essential to ensure proper Amplatzer device placement. Distortion of the atrial septum and Amplatzer device orientation occur before release but resolve on release from the delivery cable. Small residual shunts are common early, but they resolve in 6 to 12 months.

One-year follow-up of the amplatzer device to close atrial septal defects.

The early and 1-year follow-up of a single United States center using the Amplatzer atrial septal defect closure device is reported. Complete closure was documented in all patients by 1 year after device implantation.

Exposure to ionizing radiation in children undergoing Amplatzer device placement to close atrial septal defects.

To evaluate exposure to ionizing radiation during Amplatzer device occlusion, a prospective study was performed to measure surface entrance radiation dose by thermoluminescent dosimetry (TLD). Between June 1998 and April 1999, dosimetry was carried out on 12 patients with Amplatzer device occlusion of atrial septal defects (n = 10) or Fontan fenestration (n =) and 12 age-matched patients who underwent diagnostic catherization. TLD chips were placed at the posterior (PA) and right lateral (LA) chest wall as well as the thyroid (TH) and gonadal (GN) regions. The Amplatzer group had a median age of 6.4 yr (2.4-12.4 yr) and a median weight of 23.7 kg (15.6-28.9 kg), which were similar (p = NS) to those of the control group, who had a median age of 7.9 yr (3.3-16.2 yr) and a median weight of 29.9 kg (10.6-58.0 kg). Device placement was successful in 11 of 12 patients; one device was removed owing to partial obstruction of the right-upper pulmonary vein. Fluoroscopy times were also similar in the Amplatzer group (23.5 +/- 2.1 min) and the control group (16.4 +/- 3.1 min; P = NS). The measured surface entrance doses of the Amplatzer group was similar (p = NS) to those of the control group in all four regions: PA (4.96 +/- 1.88 vs. 6.07 +/- 2.16 cGy), LA (5.22 +/- 1.68 vs. 3.13 +/- 1.25 cGy), TH (0.92 +/- 0.14 vs. 0.69 +/- 0.09 cGy), and GN (0.20 +/- 0.00 vs. 0.22 +/- 0.01cGy). Fluoroscopy times and measured surface entrance doses of ionizing radiation in patients undergoing Amplatzer device occlusion are similar to those in patients undergoing routine diagnostic catheterization.

Surgical palliation of truncus arteriosus with mitral atresia and hypoplastic left ventricle.

A rare case of truncus arteriosus associated with mitral valve atresia, hypoplastic left ventricle, and intact ventricular septum is reported. Successful medical management and surgical palliation of this defect is described. The possible embryology, pertinent hemodynamics, and clinical concerns with this unusual case are discussed.

Modified Norwood operation for single left ventricle and ventriculoarterial discordance: an improved surgical technique.

BACKGROUND: Patients with univentricular hearts and ventriculoarterial discordance with potentially obstructed systemic blood flow continue to pose difficult management problems. The goals of neonatal palliative operations are to control pulmonary blood flow while avoiding pulmonary artery distortion, to relieve systemic outflow tract obstruction, and to avoid heart block. METHODS: Between January 1987 and December 1996, 38 patients with either tricuspid atresia or a double-inlet left ventricle and ventriculoarterial discordance underwent a modified Norwood procedure. Their mean age was 15 days, and their mean weight was 3.4 kg. Aortic arch anomalies were present in 92% of the patients. Morbidity and mortality statistics, intraoperative data, and postoperative echocardiograms were reviewed. RESULTS: There were 3 early deaths (7.8%) and 5 late deaths (13.1%). The actuarial survival rates at 1 month, 1 year, and 5 years were 89%, 82%, and 71%, respectively. Follow-up was complete in all children at a mean interval of 30 +/- 9 months. None of the patients had significant neoaortic valve insufficiency, and 1 patient required therapy for residual aortic arch obstruction. Nine patients (30% of the survivors) have undergone the hemi-Fontan procedure, and 18 patients (60%) successfully have undergone the Fontan procedure. CONCLUSIONS: In this patient population, we recommend the modified Norwood procedure as the neonatal palliative treatment of choice. It can be performed with acceptable early morbidity and mortality, and it improves suitability for the Fontan procedure. It reliably relieves all levels of systemic outflow tract obstruction, controls pulmonary blood flow, and avoids heart block.

Comparison of simultaneous Doppler- and catheter-derived right ventricular dP/dt in hypoplastic left heart syndrome.

Doppler estimation of RV dP/dt correlates well with micromanometer catheter-measured values in children with hypoplastic left heart syndrome. Doppler estimation of RV dP/dt is a method of quantifying RV systolic function independent of geometric assumptions, and may be a valuable method for longitudinal analysis of RV function.