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24. Midaortic syndrome in the fetus and premature newborn: a new etiology of nonimmune hydrops fetalis and reversible fetal cardiomyopathy. (Experience And Reason--Briefly Recorded)

Author

Zeltser, Ilana, Parness, Ira A., Ko, Helen, Holzman, Ian R., and Kamenir, Steven A.

Subjects
Hydrops fetalis -- Causes of, Pediatric cardiology -- Research, Cardiomyopathy -- Causes of, Cardiomyopathy -- Care and treatment, Heart diseases
Abstract

Nonimmune hydrops fetalis is the final common pathway of many conditions that ultimately result in fetal anasarca. Even after extensive evaluation, the etiology of a small percentage of cases of hydrops remains unknown. We present a case of midaortic syndrome, also known as abdominal coarctation syndrome, in a fetus with hydrops and a severe cardiomyopathy. The clinical manifestations of midaortic syndrome in this fetus and premature newborn, including malignant hypertension and reversible cardiomyopathy, are detailed. The fetal pathophysiology of midaortic syndrome remains speculative, but likely includes fetal hypertension as the cause of cardiac dysfunction. To our knowledge, this is the first report of midaortic syndrome as an etiology for nonimmune hydrops fetalis. Pediatrics 2003;111: 1437-1442; midaortic syndrome, abdominal coarctation, renal artery stenosis, hydrops fetalis, fetal cardiomyopathy., ABBREVIATIONS. NHF, nonimmune hydrops fetalis; RV, right ventricular; DA, ductus arteriosus; LV, left ventricular. Hydrops fetalis is a frequently lethal clinical syndrome manifesting as fetal anasarca that has multiple etiologies. [...]

Published
2003

30. Correlation of transthoracic echocardiography-derived pulmonary to systemic flow ratio with hemodynamically estimated left to right shunt in atrial septal defects.

Author

Faherty, Erin, Rajagopal, Hari, Lee, Simon, Love, Barry, Srivastava, Shubhika, Parness, Ira A., and Uppu, Santosh C.

Subjects
ECHOCARDIOGRAPHY, CARDIAC catheterization, SURGICAL anastomosis, PULMONARY circulation, OXIMETRY, RETROSPECTIVE studies, ATRIAL septal defects, T-test (Statistics), PEARSON correlation (Statistics), HEMODYNAMICS
Abstract

Background : Transthoracic echocardiographic (TTE) estimation of the pulmonary to systemic flow ratio (Qp/Qs) is routinely used in clinical practice and is included in the American Society of Echocardiography Guidelines. We sought to assess its real-world applicability with a particular focus on hemodynamically significant shunt lesions. Methods: Retrospective single institutional review of TTE's in patients with secundum atrial septal defect prior to cardiac catheterization (cath) from 2012 to 2018 was performed (n = 109), those with technically limited images for Qp/Qs calculation (n = 11) and those with time interval between TTE and cath >60 days were excluded (n = 14). Qp/Qs was calculated from stored clips by previously described methods and correlated with those obtained by oximetry. Patients were subdivided into two age groups <21 (Group 1) and ≥22 years (Group 2). TTE and cath methods for Qp/Qs estimation were compared using paired t-test, Pearson's correlation coefficient, and Bland-Altman plots. Results: Eighty-four subjects met inclusion criteria (age range 3-78 years). Group 1 n = 35; median age 10 years; Group 2 n = 49; median age 49 years. Transthoracic echocardiogram was performed 19.5 ± 15 days prior to cath. Mean Qp/Qs derived by cath and TTE were 2.09 ± 0.9 versus 2.54 ± 1.2 (P < 0.0001). Overall correlation was poor between the methods (r² = 0.32, P < 0.0001) and continued to be poor for Groups 1 and 2 (r² = 0.24, P = 0.003 and r² = 0.40, P < 0.0001 respectively). Bland-Altman plots demonstrated poor agreement between the predetermined limits of agreement (-0.5-1.5). Conclusion : Transthoracic echocardiography estimated Qp/Qs, although routinely utilized in clinical practice, has poor correlation and agreement with oximetry-derived Qp/ Qs. The test performs poorly in all age groups in detecting a hemodynamically significant shunt and tends to overestimate the degree of left to right shunt. [ABSTRACT FROM AUTHOR]

Published
2022
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42. Pulmonary valve-moderator band distance and association with development of double-chambered right ventricle

Author

Wong, Pierre C., Sanders, Stephen P., Jonas, Richard A., Colan, Steven D., Parness, Ira A., Geva, Tal, Van Praagh, Richard, and Spevak, Philip J.

Subjects
Heart ventricle, Right -- Physiological aspects, Ventricular septal defects -- Diagnosis, Arterial occlusions -- Diagnosis, Health
Abstract

Double-chambered right ventricle (DCRV), a form of right ventricular outflow obstruction that sometimes accompanies a ventricular septal defect (VSD), is associated with superior and rightward displacement of the septal insertion of the moderator band. It was hypothesized that this superior displacement is present and identifiable by echocardiography in patients with VSD even before right ventricular outflow tract obstruction develops. Eight patients who had a previous echocardiographic study showing a VSD alone were echocardiographically diagnosed as having DCRV. Their initial echocardiographic studies were reviewed, and superior displacement of the moderator band was quantified by measuring the distance between the pulmonary valve and moderator band, normalized to tricuspid anulus diameter. These measurements were compared with those from the initial studies of the following 3 other groups: (1) an age-matched group of 10 patients with no structural heart disease; (2) an age-matched group of 10 patients with a VSD who did not develop DCRV; and (3) a group (not age-matched) of 10 patients with VSD and DCRV in whom subpulmonary obstruction was present on the initial study. The 8 patients who eventually developed subpulmonary obstruction had significant superior displacement of the moderator band at the time of their initial echocardiogram compared with that of the 2 age-matched control groups (p Superior displacement of the moderator band is quantifiable by 2-dimensional echocardiography and may help to predict which infants with a VSD are at risk to develop subpulomonary obstruction.

Published
1991

43. The Authors Reply

Author

Lorber, Richard, primary, Srivastava, Shubhika, additional, Wilder, Travis J., additional, McIntyre, Susan, additional, DeCampli, William M., additional, Williams, William G., additional, Frommelt, Peter, additional, Parness, Ira A., additional, Blackstone, Eugene H., additional, Jacobs, Marshall, additional, Mertens, Luc, additional, Brothers, Julie, additional, and Herlong, J. René, additional

Published
2016
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45. Anomalous Aortic Origin of Coronary Arteries in the Young

Author

Lorber, Richard, primary, Srivastava, Shubhika, additional, Wilder, Travis J., additional, McIntyre, Susan, additional, DeCampli, William M., additional, Williams, William G., additional, Frommelt, Peter C., additional, Parness, Ira A., additional, Blackstone, Eugene H., additional, Jacobs, Marshall L., additional, Mertens, Luc, additional, Brothers, Julie A., additional, and Herlong, J. René, additional

Published
2015
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46. Application of Virtual Three-Dimensional Models for Simultaneous Visualization of Intracardiac Anatomic Relationships in Double Outlet Right Ventricle

Author

Farooqi, Kanwal M., primary, Uppu, Santosh C., additional, Nguyen, Khanh, additional, Srivastava, Shubhika, additional, Ko, H. Helen, additional, Choueiter, Nadine, additional, Wollstein, Adi, additional, Parness, Ira A., additional, Narula, Jagat, additional, Sanz, Javier, additional, and Nielsen, James C., additional

Published
2015
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