Impact of altered loading conditions on ventricular performance in fetuses with congenital cystic adenomatoid malformation and twin–twin transfusion syndrome

Department of Pediatrics, Cardiology Division, Fetal Heart Program, Children's Hospital of Philadelphia, Philadelphia, PA 19050, USA.
Ultrasound in Obstetrics and Gynecology (Impact Factor: 3.85). 07/2007; 30(1):40-6. DOI: 10.1002/uog.4032
Source: PubMed


In the fetus with a structurally normal heart, two conditions--giant chest mass, such as congenital cystic adenomatoid malformation (CCAM), and twin-twin transfusion syndrome (TTTS)--alter ventricular loading conditions and may result in cardiovascular compromise. The aim of this study was to elucidate the mechanism of cardiovascular dysfunction by comparing geometry-independent, Doppler flow-derived measures of ventricular performance in fetuses with altered loading conditions vs. those in normal fetuses.
Doppler flow-derived measures of myocardial performance index (MPI) as described by Tei, ventricular ejection force as described by Isaaz, and combined cardiac output (CCO) were obtained by echocardiography in fetuses with a normal cardiovascular system (n = 76) or CCAM (n = 36) and fetal partners with TTTS (n = 22).
In the CCAM group, systolic performance as evidenced by the ejection forces was preserved, right ventricular (RV) MPI was increased and CCO diminished, suggesting diastolic dysfunction and poor filling secondary to cardiac compression and a tamponade effect. In TTTS, recipient twins exhibited greater left ventricular (LV) ejection forces and higher CCO than donor twins, and had abnormal RV and LV MPI, reflecting increased preload, preserved left systolic performance, but diastolic dysfunction. Donor twins had diminished ejection forces and CCO in comparison with normal controls and recipient partners, reflecting hypovolemia.
In both CCAM and recipient twins of the TTTS, diastolic dysfunction plays a significant role in the pathophysiology of each disorder and precedes changes in systolic performance. Measures of ventricular performance can help elucidate poorly understood mechanisms of cardiovascular compromise in the developing fetus.

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Available from: Robert Douglas Wilson, Apr 07, 2015
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    • "Accordingly, speckle-tracking-derived measurements of strain and strain rate, although difficult to perform, show decreased strain in the right ventricle of recipient fetuses of TTTS [25]. In contrast to the lower contractility and to earlier reports that did not show differences in cardiac output between donors and recipients [22] [26], two recent series in relatively large cohorts of recipient fetuses have shown a moderate increase in cardiac output when corrections were made for fetal weight [23] [27]. This finding clearly fits in with the volume overload theory. "
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    ABSTRACT: Twin-to-twin transfusion syndrome is a severe complication occurring in 10% of monochorionic twin pregnancies. The disease is usually explained as due to an intrauterine imbalance in intertwin blood exchange, which leads to a volume depleted-donor twin and an overfilled recipient twin. The recipient has signs of cardiac dysfunction, which can be measured using echocardiography or blood and amniotic fluid derived biomarkers. Whereas cardiac dysfunction typically progresses in pregnancies treated with amniodrainage, it usually disappears within a few weeks after fetoscopic laser coagulation of the connecting intertwin anastomoses. Nevertheless, recipients remain at a increased risk of pulmonary stenosis. In this paper, we summarize the cardiac alterations in twin-to-twin transfusion syndrome, describe the changes seen after fetal therapy, list the newly proposed staging systems based on fetal cardiac function, and make recommendations about the use of fetal echocardiography in the evaluation and followup of pregnancies complicated by twin-to-twin transfusion syndrome.
    International Journal of Pediatrics 08/2010; 2010(9). DOI:10.1155/2010/379792
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    ABSTRACT: To compare fetal cardiac output (CO) in donor and recipient twins of twin-twin transfusion syndrome (TTTS) pregnancies after laser therapy with that of monochorionic twins without TTTS and normal singletons. In a longitudinal, prospective study, we sonographically assessed fetal CO in donors (n = 10) and recipients (n = 10) with TTTS after fetoscopic laser therapy, in monochorionic twins without TTTS (n = 20) and in normal singleton pregnancies (n = 20). The fetal CO of TTTS twins was determined 1 day and 1 week after laser treatment, and from then on every 2-4 weeks until birth. Twins without TTTS were examined biweekly until birth. Singletons were examined twice, with an 8-week interval, at different gestational ages between 17 and 35 weeks. Absolute CO increased exponentially with advancing gestational age (P < 0.0001), and was significantly related to fetal weight in all groups (P < 0.0001). The median CO/kg in donors after laser therapy, recipients after laser therapy and non-TTTS monochorionic twins was significantly higher than that in singletons (all P < 0.001). Median CO/kg in donors after laser therapy, recipients after laser therapy, and non-TTTS monochorionic twins was not significantly different between groups. Monochorionic twins with TTTS have higher CO after laser therapy than normal singletons.
    Ultrasound in Obstetrics and Gynecology 11/2008; 32(6):807-12. DOI:10.1002/uog.6230 · 3.85 Impact Factor
  • Prenatal Diagnosis 01/2009; 29(1):6-10. DOI:10.1002/pd.2162 · 3.27 Impact Factor
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