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: 3.14). 08/2007; 30(1):40-6. DOI: 10.1002/uog.4032
Source: PubMed

ABSTRACT 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.

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: We describe the hemodynamic changes observed in fetuses with extra-cardiac conditions such as intra-uterine growth restriction (IUGR), tumors, twin-twin transfusion syndrome (TTTS), congenital infections and in fetuses of mothers with diabetes. In most fetuses with mild extra-cardiac disease, the alterations in fetal cardiac function remain subclinical. Cardiac function assessment has however helped us to achieve a better understanding of the pathophysiology of these diseases. In fetuses at the more severe end of the disease spectrum, functional echocardiography may help in guiding clinical decision making regarding the need for either delivery or fetal therapy. The growth restricted fetus represents a special indication for routine cardiac function assessment, as in utero hemodynamic changes may help optimize the timing of delivery. Moreover, in IUGR, the altered hemodynamics cause cardiovascular remodeling which can result in an increased risk of postnatal cardiovascular disease. This article is protected by copyright. All rights reserved.
    Prenatal Diagnosis 10/2013; · 2.68 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Twin-to-twin transfusion syndrome (TTTS) affects about 10% of monochorionic pregnancies and develops when uncompensated unidirectional blood flow from one twin ('donor') to the other ('recipient') causes circulatory imbalance. The cardiac effect of the underlying hypervolemia or endocrine dysregulation, or both, manifests in the recipient as echocardiographic findings of the syndrome-related cardiomyopathy. The Quintero staging system for TTTS has recently been questioned, because more refined measurement of cardiac function may improve evaluation of disease severity and prediction of outcome. Much has been done to increase survival and diminish the cardiac morbidity associated with TTTS. The purpose of this paper was to review the literature on cardiac morbidity associated this syndrome.
    Journal of Perinatal Medicine 01/2012; 40(2):107-14. · 1.43 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin-twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.
    Circulation 04/2014; · 14.95 Impact Factor