Ann Kavanaugh-McHugh

Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease

A member of the Tennessee state legislature recently proposed a bill that would mandate all newborn infants to undergo pulse oximetry screening for the purpose of identifying those with critical structural heart disease before discharge home. The Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Defects was convened on September 29, 2005. This group reviewed the current medical literature on this topic, as well as data obtained from the Tennessee Department of Health, and debated the merits and potential detriments of a statewide screening program. The estimated incidence of critical congenital heart disease is 170 in 100000 live births, and of those, 60 in 100000 infants have ductal-dependent left-sided obstructive lesions with the potential of presentation by shock or death if the diagnosis is missed. Of the latter group, the diagnosis is missed in ∼9 in 100 000 by fetal ultrasound assessment and discharge examination and might be identified by a screening program. Identification of the missed diagnosis in these infants before discharge could spare many of them death or neurologic sequelae. Four major studies using pulse oximetry screening were analyzed, and when data were restricted to critical left-sided obstructive lesions, sensitivity values of 0% to 50% and false-positive rates of between 0.01% and 12% were found in asymptomatic populations. Because of this variability and other considerations, a meaningful cost/benefit analysis could not be performed. It was the consensus of the task force to provide a recommendation to the legislature that mandatory screening not be implemented at this time. In addition, we determined that a very large, prospective, perhaps multistate study is needed to define the sensitivity and false-positive rates of lower-limb pulse oximetry screening in the asymptomatic newborn population and that there needs to be continued partnering between the medical community, parents, and local, state, and national governments in decisions regarding mandated medical care.

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.

Pericardial effusion and cardiac tamponade in pediatric stem cell transplant recipients.

Summary:Pericardial effusion and cardiac tamponade is a rarely reported complication following stem cell transplant (SCT). The incidence among pediatric SCT recipients is not well defined. To assess the frequency of clinically significant pericardial effusions, we retrospectively examined clinically significant cardiac effusions at our center. Between January of 1993 and August 2004, clinically significant pericardial effusions were identified in nine of 205 patients (4.4%). The median age at the time of transplant was 9 years (range 0.6–18 years) and seven received an allogeneic transplant. All nine had normal cardiac function prior to transplant. The effusion developed at a median of 30 days (range 18–210 days). All allogeneic recipients had acute or clinically extensive graft-versus-host disease (GVHD) at the time the effusion was diagnosed. Seven patients (78%) required pericardiocentesis or surgical creation of a pericardial window. No patient died as a complication of the effusion or the therapeutic procedures. Clinically significant pericardial effusions are more common than previously reported in pediatric SCT recipients. Acute and chronic GVHD is an associated factor.

Modified Norwood operation for hypoplastic left heart syndrome

BACKGROUND We examined early results in infants with hypoplastic left heart syndrome undergoing the Norwood operation with perioperative use of inhaled nitric oxide and application of extracorporeal membrane oxygenation. METHODS Medical records were reviewed retrospectively. RESULTS Between April 1997 and March 2001, 50 infants underwent a modified Norwood operation for hypoplastic left heart syndrome. Mean age at operation was 7.5 +/- 5.7 days, and mean weight was 3.1 +/- 0.5 kg. Five infants had a delayed operation because of sepsis. The mean diameter of the ascending aorta by echocardiography was 3.6 +/- 1.8 mm. Ductal cannulation was used to establish cardiopulmonary bypass in all patients. Mean circulatory arrest time was 39.4 +/- 4.8 minutes. The size of the pulmonary-systemic shunt was 3.0 mm in 6 infants, 3.5 mm in 37, and 4.0 mm in 7. Infants with persistent hypoxia (partial pressure of oxygen < 30 mm Hg) received nitric oxide after they were weaned from cardiopulmonary bypass. Extracorporeal membrane oxygenation was initiated in 8 infants in the pediatric intensive care unit primarily for low cardiac output and in 8 in the operating room because of the inability to separate them from cardiopulmonary bypass. The 30-day mortality rate was 22% (11 of 50 patients), and the hospital mortality rate was 32% (16 of 50 patients). Mean follow-up was 17 months. Ten patients (20%) underwent stage-two repair, with one operative death. One survivor had a Fontan procedure, and 2 underwent heart transplantation, with one death. CONCLUSIONS Early application of extracorporeal membrane oxygenation for hemodynamic instability and selective use of nitric oxide for persistent hypoxia in the immediate postoperative period may improve survival of patients with hypoplastic left heart syndrome. Renal failure requiring hemofiltration during extracorporeal membrane oxygenation (p < 0.05) and cardiopulmonary arrest in the pediatric intensive care unit (p < 0.05) were predictors of hospital mortality.

Endocarditis Caused by Group A β-Hemolytic Streptococcus in an Infant: Case Report and Review

Acute bacterial endocarditis in the absence of underlying heart disease is rare. We report the occurrence of endocarditis caused by group A beta-hemolytic Streptococcus (GABHS), following varicella, in a 5-month-old child without heart disease. In addition to this child, seven other children with endocarditis caused by GABHS have been reported since 1966, six of whom did not have preexisting heart disease. In one of these children, GABHS endocarditis was preceded by varicella. These cases indicate that GABHS is capable of causing endocarditis in the absence of heart disease, and they provide further evidence that varicella is an important risk factor for invasive GABHS infections.

Low incidence of significant valvar insufficiency following retrograde aortic radiofrequency catheter ablation in young patients.

The incidence of significant valvar insufficiency at late (>6 month) follow-up was retrospectively evaluated in 27 young patients (age 4.0–18.0 years) undergoing 29 ablation procedures via the retrograde aortic approach for left-sided accessory connections in whom pre-ablation and post-ablation echocardiograms were available for review. Valvar insufficiency was graded using color flow techniques as absent, trivial, mild, moderate, or severe by blinded reviewers. Ablation was acutely successful via the retrograde approach in 25 of 29 procedures among these 27 patients. Successful ablation was ultimately achieved in all 27 patients.At baseline, 7 patients had evidence of trivial or mild mitral insufficiency, and no patient had aortic insufficiency. Three patients had evidence of impaired left ventricular systolic performance in the presence of manifest pre-excitation. At follow-up, pre-existing mitral insufficiency resolved in 5/7 patients, and persisted in 2 patients. New mitral insufficiency was evident in 3 patients, and new aortic insufficiency was transiently evident in 1 patient following ablation (all trivial). Institutional experience (mean rank 10 cases vs. 33 cases, p < .0005), and lower patient weight (29.7 vs. 56.3 kilograms, p = .01) were the only factors associated with the development of new valvar insufficiency. Valvar insufficiency could not be detected by careful auscultation in any patient and was deemed clinically insignificant in all patients.We conclude that ablation of left-sided accessory connections can be performed via the retrograde aortic approach without creating clinically significant valvar insufficiency.

Pediatric prenatal diagnosis of congenital heart disease.

Purpose of review Fetal cardiology is a rapidly evolving field. Imaging technology continues to advance as do approaches to in-utero interventions and care of the critically ill neonate, with even greater demand for improvement in prenatal diagnosis of congenital heart disease (CHD) and arrhythmias. Recent findings Reviewing the advances in prenatal diagnosis of CHD in such a rapidly developing field is a broad topic. Therefore, we have chosen to focus this review of recent literature on challenges in prenatal detection of CHD, challenges in prenatal counseling, advances in fetal arrhythmia diagnosis, and potential benefits to patients with CHD who are identified prenatally. Summary As methods and tools to diagnose and manage CHD and arrhythmias in utero continue to improve, future generations will hopefully see a reduction in both prenatal and neonatal morbidity and mortality. Prenatal diagnosis can and should be used to optimize location and timing of delivery and postnatal interventions. Video abstract http://links.lww.com/MOP/A21

A Case of an Infant with Flail Tricuspid Valve Due to Spontaneous Papillary Muscle Rupture: Was Neonatal Lupus the Culprit?

A 3-month-old infant presented in extremis with a flail tricuspid valve. The authors theorized that the genesis of her papillary muscle rupture was perinatal ischemia compounded by worsening pulmonary valvular stenosis leading to excessive fiber tension. Her underlying diagnosis of autoimmune-mediated heart block with endocardial fibroelastosis and prenatal glucocorticoid steroid treatment represents potentiating factors.

Transesophageal echocardiography in pediatric congenital heart disease.

The uses of transesophageal echocardiography have expanded dramatically over the last decade. With advances in technology, this imaging modality has become readily available for evaluation of the complex pediatric population with congenital heart disease. This article discusses the many uses of transesophageal echocardiography in this population, in the outpatient setting, in the peri-operative period, and in the cardiac catheterization laboratory.

Oximetry and Capnography During Sedation For Transesophageal Echocardiography

procedures such as arterial blood gas analysis. Additional applications of ETC02 monitoring may include patient monitoring during conscious sedation, documentation of endotracheal tube placement during intubation, and as a means to judge the effectiveness of cardiopulmonary resuscitation. We have found ETC02 monitoring a valuable adjunct to other monitors during sedation, both in and outside the operating room. Although the use of pulse oximetry is recommended because hypoxemia is significantly more detrimental than hypercarbia, there