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.

Estimated cardiac output and cardiovascular profile score in fetuses with high cardiac output lesions.

OBJECTIVE: High cardiac output lesions are associated with an increased risk of fetal death, largely as a result of cardiac failure and hydrops fetalis. The cardiovascular profile score (CVPS) has been used to characterize cardiovascular wellbeing, and has been linked to fetal outcomes in other conditions. We aimed to test the hypothesis that elevated combined cardiac output (CCO) in fetuses with high output lesions may be associated with worsening cardiovascular status, as evidenced by a lower CVPS. METHODS: A retrospective review was performed of fetuses with high cardiac output lesions that underwent echocardiography between July 2006 and November 2010. Diagnoses included sacrococcygeal teratoma, placental chorioangioma and vein of Galen aneurysm. Fetal echocardiographic evaluation included assessment of CVPS, as well as Doppler/two-dimensional estimation of CCO, indexed to estimated fetal weight (CCOi). The relationship between CCO and CVPS was assessed. RESULTS: A total of 35 fetuses were studied: 27 had sacrococcygeal teratoma, seven had chorioangioma and one had vein of Galen aneurysm. There was a significant inverse relationship between mean logCCOi and CVPS (r2 = 0.48, P = 0.008). Of 31 patients with clinical outcome data, 10 experienced either in-utero demise or intervention; 80% of these fetuses had a CVPS of < 8. CONCLUSIONS: There is an inverse relationship between CCO and CVPS in the fetus with high cardiac output lesions. As a measure of fetal cardiovascular wellbeing in this population, the CVPS may be a useful tool for stratifying risk and for selection for intervention in these fetuses.

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.

Standardization of amniotic fluid leptin levels and utility in maternal overweight and fetal undergrowth.

OBJECTIVE: Leptin is an adipokine that regulates energy homeostasis. The objective of this study was to establish a gestational age-specific standard for amniotic fluid leptin (AFL) levels and examine the relationship between AFL, maternal overweight and fetal growth restriction. STUDY DESIGN: Amniotic fluid was obtained at mid-gestation from singleton gravidas, and leptin was quantified using enzyme-linked immunosorbent assay. Amniotic fluid samples from 321 term pregnancies were analyzed. Clinical data, including fetal ultrasound measurements and maternal and infant characteristics, were available for a subset of patients (n=45). RESULTS: The median interquartile range AFL level was significantly higher at 14 weeks' gestation (2133 pg ml(-1) (1703 to 4347)) than after 33 weeks' gestation (519 pg ml(-1) (380 to 761), P trend<0.0001), an average difference of 102 pg ml(-1) per week. AFL levels were positively correlated with maternal pre-pregnancy body mass index (BMI) (r=0.36, P=0.03) adjusting for gestational age at measurement, but were not associated with fetal growth. CONCLUSIONS: AFL levels are higher at mid-gestation than at late gestation, and are associated with maternal pre-pregnancy BMI.

Left bundle branch block in infants with dilated cardiomyopathy conveys a poor prognosis.

We describe three infants <3 months of age seen consecutively with dilated cardiomyopathy who presented initially with left bundle branch block on the surface 12-lead electrocardiogram. Each infant subsequently had a poor outcome: two died and one required heart transplantation. These results suggest that the presence of left bundle branch block on the 12-lead electrocardiogram conveys a poor prognosis in infants with dilated cardiomyopathy.

Purpura fulminans associated with Streptococcus pneumoniae infection in a child.

BACKGROUND: Neisseria meningitidis is the most frequent isolate associated with purpura fulminans in children. Although Streptococcus pneumoniae infection has been associated with purpura fulminans, with the exception of one adult, it has only been reported in immunocompromised hosts. PURPOSE: We report an apparently previously healthy child who presented with purpura fulminans associated with pneumococcal meningitis. METHODS: Case report and review of the medical literature from September 1966 to June 1997, using a MEDLINE search. CONCLUSION: While systemic pneumococcal infection is common in childhood, progression to purpura fulminans does not typically occur in overtly healthy children. Our patient illustrates that invasive pneumococcal infection should be considered and empirically treated in a child who presents with purpura fulminans, even in the absence of preexisting functional or anatomic asplenia.