Cyrus Samai

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era A Multicenter Study

Background— Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results— Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5–21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1–1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4–6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1–6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion— In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

Pediatric Appropriate Use Criteria for Echocardiography: Implications for Clinical Practice

The first pediatric Appropriate Use Criteria (AUC) were recently published by the American College of Cardiology.1 This document consists of common clinical scenarios for which a transthoracic echocardiogram is ordered in outpatient settings. Echocardiography is the most common imaging modality used in the outpatient setting to assess children with suspected heart disease. However, it is known that echocardiography is not cost-effective or high yield for some common indications such as chest pain, syncope, or murmurs.2–4 The AUC have been developed to guide physician decision-making for appropriate testing and to improve resource utilization. In addition to pediatric cardiologists, a wide variety of providers including pediatricians, family practitioners, and other pediatric subspecialists order this imaging test. Therefore, this document has a very broad audience. Although appropriateness criteria may be a new concept in the pediatric world, adult cardiologists have been using these standards for various invasive and noninvasive procedures for almost a decade.5 In the first half of 2000, because of the dramatic augmentation of cardiac imaging, concerns were raised about excessive spending on these tests compared with other areas of health care. Consequently, health plans started adopting a variety of strategies to manage utilization, such as preauthorization. Concurrently, the American College of Cardiology Foundation established an AUC Task Force to guide appropriate testing. The purpose of the AUC was to guide physicians about “when and how often” to order a given procedure in an individual patient on the basis of current scientific evidence and health care resources. The overall goals of the AUC are to … Address correspondence to Ritu Sachdeva, MBBS, Division of Pediatric Cardiology, Emory University School of Medicine, 1405 Clifton Rd NE, Atlanta, GA 30322-1062. E-mail: sachdevar{at}kidsheart.com

Primary congenital infantile fibrosarcoma of the heart: the first confirmed case.

We believe this case represents the first confirmed congenital infantile fibrosarcoma (CIFS) of the heart. We describe an infant with congenital infantile fibrosarcoma of the heart who was treated with chemotherapy and who had a life-threatening complication develop that was successfully corrected by surgery. This demonstrates an unusual presentation, remarkable imaging, and diagnostic and treatment dilemmas. More significantly, this emphasizes the importance of a multidisciplinary approach for successful treatment of congenital infantile fibrosarcoma.

Temporal Trends in Utilization of Transthoracic Echocardiography for Common Outpatient Pediatric Cardiology Diagnoses over the Past 15 Years

Background: No data exist regarding the temporal trends in utilization of transthoracic echocardiography (TTE) in an outpatient pediatric cardiology setting. This study evaluates the trends in utilization of TTE for common diagnoses known to have low diagnostic yield and the factors influencing these trends. Methods: Patients evaluated at our pediatric cardiology clinics from January 2000 to December 2014 and discharged with final diagnoses of innocent murmur, noncardiac chest pain, benign syncope, and palpitations were included. Variables collected retrospectively included patient age, sex, insurance type, distance from clinic, and ordering physicians years of experience since fellowship. Results: Of the 74,881 patients seen by 35 physicians, 36,053 (48.1%) had a TTE. The TTE rates increased from the beginning of 2000 to the end of 2004 (5.2% per year; P < .001) and then steadily declined until the end of 2014 (1.6% per year; P < .001). Utilization for noncardiac chest pain remained the highest, and use in infants increased significantly during the study period (P < .001). After adjusting for all other factors, the following variables were associated with higher TTE utilization: younger age, males, Medicaid insurance, increased distance from clinic, and being seen by less experienced physicians. Temporal trends persisted after adjusting for all these factors. Conclusions: After an initial surge in TTE utilization from 2000 to 2004, there was a steady decline. This study identifies some important factors influencing these trends. This information could help design quality interventions, but additional factors need to be explored since the trends persist despite adjusting for these factors.

Pulmonary Hypertension in Kawasaki Disease

This report describes the case of two pediatric patients who demonstrated echocardiographic evidence of pulmonary hypertension (PH) during the acute phase of Kawasaki disease. The etiology of PH development in this setting is currently unknown, but the authors hypothesize that pulmonary vasculitis may play a significant role. Fortunately, the PH appeared to be self-limited and resolved in both cases with routine treatment of Kawasaki disease.

Lower Extremity Edema in a Child Due to Pectus Excavatum

A previously healthy 11-year-old girl was referred for pediatric cardiology evaluation because of the development of progressive bilateral lower extremity swelling over the course of 2 years. Her prior workup had included a negative result for proteinuria and a negative ultrasound for deep venous thrombosis. On physical examination, in addition to her edema, she was found to have a severe pectus excavatum deformity, which prompted cardiac magnetic resonance imaging. This study demonstrated compression of the inferior vena cava. She underwent uneventful pectus repair with use of a modified Ravitch procedure and experienced complete resolution of her lower extremity edema.

Maternal Antibody-Associated Fetal Second-Degree Heart Block and Atrial Flutter: Case Report and Review

Exposure to maternal anti-Ro (SS-A) and anti-La (SS-B) antibodies is a well-described risk factor for the development of fetal atrioventricular (AV) block. The role of maternal fluorinated steroids in the treatment and prevention of antibody-mediated fetal AV block is controversial. Fetal atrial flutter has rarely been described in association with maternal antibodies. This report describes a case of fetal exposure to maternal anti-Ro antibodies with associated second-degree AV block and atrial flutter. Interestingly, the reported patient had 2:1 AV conduction during both normal atrial rates (consistent with AV node conduction disease) and episodes of flutter (consistent with physiologic AV node functionality). The fetus was treated with transplacental digoxin and dexamethasone, which resolved both rhythm disturbances. The case report is followed by a brief discussion of AV block and atrial flutter associated with maternal antibody exposure.

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era

Background— Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results— Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5–21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1–1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4–6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1–6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion— In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current EraCLINICAL PERSPECTIVE: A Multicenter Study

Background— Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results— Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5–21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1–1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4–6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1–6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion— In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current EraCLINICAL PERSPECTIVE

Background— Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results— Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5–21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1–1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4–6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1–6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion— In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.