Carolyn A. Altman

Respiratory syncytial virus in patients with congenital heart disease: a contemporary look at epidemiology and success of preoperative screening.

Abstract. Awareness of respiratory syncytial virus (RSV) as a serious pathogen in the child with congenital heart disease is increasing. We studied the impact of RSV lower respiratory tract disease on patients in a large academic pediatric cardiology practice. We found that RSV disease necessitating hospitalization occurs in congenital heart disease patients well into the second year of life. Although pulmonary hypertension remains a significant risk factor for morbidity in these patients, it does not appear to be as much of a factor as in the past. By implementing a nasopharyngeal RSV enzyme-linked immunoassay screening of young patients prior to cardiac surgery we found a reduction in community-acquired postoperative RSV disease. We postulate this will lead to a reduction in nosocomial disease in the postoperative care unit.

Risk Factors for Neo-Aortic Root Enlargement and Aortic Regurgitation Following Arterial Switch Operation

The objectives of this study were to evaluate changes in dimension of the neo-aortic annulus, aortic root, and aortic anastomosis following arterial switch operation (ASO) and to identify risk factors for developing abnormal neo-aortic root enlargement and aortic regurgitation (AR). Prior studies report development of neo-aortic root dilatation and AR in a small subset of patients after ASO. Predisposing factors for neo-aortic root dilatation and development of moderate/severe AR are poorly understood. We performed a retrospective review of all patients with d-transposition of the great arteries (d-TGA) or double-outlet right ventricle with subpulmonary ventricular septal defect (VSD) who underwent ASO from May 1986 to January 2001. Serial echocardiograms were reviewed to measure neo-aortic annulus, root, and anastomosis diameter (z scores) and to determine progression of AR. Potential risk factors were assessed for developing neo-aortic root enlargement and AR. There were 119 patients (44 female and 75 male): 73 patients had simple d-TGA, 36 had d-TGA with ventricular septal defect, and 10 had a Taussig–Bing heart. The median duration of follow-up was 65 months (range, 12–180). The median neo-aortic root (z = 0.55 ± 2.2; p < 0.01) and aortic annulus dimensions (z = 1.57 ± 1.75; p < 0.01) were significantly increased over the study period. Aortic anastomosis diameter correlated with growth of the ascending aorta (z = 0.55 ± 1.24). Development of severe neo-aortic root enlargement was associated with prior pulmonary artery (PA) banding (p < 0.01), the presence of a VSD (p = 0.03), and Taussig–Bing anatomy (p < 0.01) but was independent of coronary arterial anatomy, coronary arterial transfer technique, or associated lesions (p > 0.05). At latest follow-up, there was no or trivial AR in 88 patients, mild AR in 29 patients, and moderate to severe AR in 3 patients. Risk factors for developing mild or worse AR included severe or rapid neo-aortic root dilatation (p < 0.01). Only 3 patients required surgical intervention for AR. Despite the significant prevalence of neo-aortic root enlargement at intermediate follow-up after ASO, there is a low incidence of significant AR. Prior PA banding, the presence of VSD, and Taussig–Bing anatomy are risk factors for severe root enlargement. Surgical intervention for AR was rare (2%), however, serial surveillance of such patients is vital to monitor for neo-aortic root enlargement and potential aortic valve dysfunction.

Outcome of pregnancy following intervention for coarctation of the aorta

There are limited data regarding the outcome of pregnancy in women after intervention for coarctation of the aorta (CoA). The Texas Childrens Hospital Cardiac Database was used to identify female patients with CoA born before 1980 who had undergone balloon angioplasty or surgery. Patients with Turners syndrome and cyanotic congenital heart disease were excluded. A chart review and telephone interview were performed. Data collected included age at intervention, type of intervention, the need for reintervention, functional status, number of pregnancies, and pregnancy outcomes. Seventy-four patients met our criteria and we were able to contact 52. Eighteen patients (39%) were pregnant a total of 36 times. There were 3 spontaneous and 4 elective abortions. Preeclampsia complicated 4 pregnancies in 3 women (17% of primigravidas). One patient had systemic hypertension. Eleven infants were delivered by Cesarean section. There were 29 births, with an average weight of 3.0 kg. There were 5 preterm births, 4 to a teenage mother. Only 1 child (3%) had a congenital heart defect. Thus, in women with an arm-to-leg blood pressure gradient of <20 mm Hg after CoA repair, pregnancy is successful. The occurrence of congenital heart disease in the offspring was 3%. Preeclampsia was similar to that in the general population.

Family-Member Presence During Interventions in the Intensive Care Unit: Perceptions of Pediatric Cardiac Intensive Care Providers

OBJECTIVE. Should family members be present during interventions in an ICU? This question is a source of debate among health care providers. We propose to define perceptions and practice regarding family-member presence during ICU interventions from a multidisciplinary group of pediatric cardiac intensive care providers. METHODS. A 20-question survey was created and distributed to attendees of the 2004 Pediatric Cardiac Intensive Care Symposium, 1 year after the meeting. Interventions were defined as noninvasive (team rounds), invasive (tracheal intubation, central/arterial line placement, chest tube placement, or pericardiocentesis), or extremely invasive (cardiopulmonary resuscitation). RESULTS. A total of 211 surveys (145 physicians and 66 nonphysicians) were completed. Of all responders, the majority believe family members have a right to be present during cardiopulmonary resuscitation (75%), team rounds (77%), and invasive procedures (57%). Sixty-five percent of respondents encounter families that frequently request to be present for team rounds. However, the majority of respondents encounter families that rarely request to be present during invasive procedures (69%) and cardiopulmonary resuscitation (73%). Many providers practice in ICUs where family-member presence is allowed; 64% allow family members to attend team rounds. Some of the concerns providers have regarding family-member presence in the ICU include family-member presence causing stress to the provider during invasive procedures along with distractions and nervousness among the team during cardiopulmonary resuscitation. The majority of providers predict family-member presence during cardiopulmonary resuscitation would not increase medicolegal concerns. CONCLUSIONS. Most respondents, nonphysicians more than physicians, believe that family members have a right to be present during all ICU interventions. The majority of respondents encounter families that frequently request to be present for team rounds. However, the majority of respondents encounter families that rarely request to be present during invasive procedures and cardiopulmonary resuscitation. Most respondents believe family-member presence during cardiopulmonary resuscitation would not increase medicolegal concerns.

Safety and efficacy of intravenous contrast imaging in pediatric echocardiography

This study was performed to determine the safety and efficacy of intravenous contrast echocardiography in children attending a tertiary cardiac center. This was a prospective study to evaluate the use of Optison contrast agent in children with severely limited transthoracic echocardiographic windows. Twenty children (median age, 15 years; range, 9–18) underwent fundamental imaging (FI), harmonic imaging (HI), and HI with intravenous contrast (Optison FS-069). Endocardial border delineation was determined based on a visual qualitative scoring system (0, none: 4, excellent). Endocardial border definition was significantly improved in all patients using contrast echocardiography (FI vs Optison, p < 0.001 for each). Improved border definition was most dramatic in the apical and left ventricular (LV) free wall regions. Left ventricular ejection fraction (LVEF) was measurable in 20 patients (100%) using contrast compared to 11 (55%) with FI or HI (p < 0.05). The echocardiographic diagnosis was correctly delineated in 1 patient with a severely dyskinetic LV segment only with use of intravenous contrast and HI. No patients suffered adverse hemodynamic effects, changes in taste, or flushing episodes. Three patients experienced transient headaches. Intravenous contrast echocardiography offers an additional tool in evaluating children with very poor transthoracic echocardiographic windows. Such a strategy increases diagnostic accuracy and allows accurate LVEF determination. Adverse hemodynamic effects related to intravenous contrast are exceedingly rare.

Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors

PURPOSE The aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors. METHODS The records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively. Hydrops was defined as accumulation of fluid in 2 or more compartments. RESULTS Of fetuses with high-risk lung mass, hydrops was identified in 46% (11/24). Fetuses with hydrops and an abnormal echocardiogram (n = 8) demonstrated poor survival without fetal surgery (13%) compared with 100% survival in fetuses with hydrops and a normal echocardiogram (n = 3; P = .02). Of 21 fetuses with vascular tumor (11 sacrococcygeal and 8 cervical teratomas; 2 hemangioendotheliomas), hydrops was identified in 29% and an abnormal echocardiogram in 57%. All fetuses with hydrops had an abnormal echocardiogram and either died (n = 5) or required fetal surgery (n = 1). However, all fetuses with abnormal echocardiograms alone (n = 7) survived without fetal intervention. CONCLUSIONS For fetuses with lung mass, an abnormal echocardiogram in the setting of hydrops is the best predictor of mortality and need for fetal surgery. For fetuses with vascular tumor, hydrops in the setting of high-output physiology best predicts demise and need for fetal surgery.

Severe clinical phenotype due to an interstitial deletion of the short arm of chromosome 1: A brief review

We report on a newborn girl with malformed ears, bilateral cleft lip and cleft palate, complex congenital heart disease, absent left thumb, and rib abnormalities. Cytogenetic analysis demonstrated a de novo interstitial deletion of the short arm of chromosome 1 [46,XX,del(1)(p21p22.3)]. Reports of interstitial deletions on the short arm of chromosome 1 are rare. However, when comparing this patients phenotype to others with deletions of 1p, we found that the current case was much more severely affected than previously reported cases.

Noninvasive diagnostic features of partial absence of the pericardium

Abstract Congenital absence of the pericardium usually involves the left side of the heart, and may be partial or complete. This condition is usually an incidental finding on a chest xray, in the operating room or at autopsy.1 Patients are generally asymptomatic, but dyspnea, syncope or atypical chest pain can occur.2,3 Much more rarely, sudden death may ensue if the left atrium or ventricle incarcerates through a partial defect.4 Surgical repair is therefore generally recommended when a partial pericardial defect is diagnosed. We report the case of an asymptomatic patient in whom the diagnosis was suggested radiographically and confirmed by cross-sectional echocardiography and magnetic resonance imaging. The findings on cross-sectional echocardiography and magnetic resonance imaging are compared with cineangiography.

Fetal cardiac malposition: incidence and outcome of associated cardiac and extracardiac malformations.

Cardiac malposition is a rare but important finding when detected on fetal ultrasound. The purpose of this study was to evaluate the incidence of fetal cardiac malposition, associated abnormalities, and clinical outcome in a tertiary-care medical center. Records of fetuses (1993 to 2006) with dextroposition, dextrocardia, mesocardia, ectopia cordis, or heterotaxy were reviewed. The presence of congenital heart disease (CHD), extracardiac anomalies, and outcome were noted. Cardiac malposition was present in 101 fetuses among a total of 3313 (3%) pregnancies. In 78 (78%) patients, the heart was positioned in the right hemithorax. Of those, 26 (33%) had dextrocardia (CHD = 21), and 52 (67%) had dextroposition (CHD = 14). Sixteen (16%) patients had mesocardia (CHD = 8), and 7 (7%) had ectopia cordis (CHD = 6). The majority (58%) of fetal cardiac malposition was caused by intrathoracic masses. Concomitant CHD occurred in 50%. Outcome was available in 97%. The perinatal and neonatal mortality rate was 30%; the elective termination rate was 4%. Patients with CHD had higher mortality rates. The highest mortality rates occurred in ectopia cordis and combined disease of diaphragmatic hernia and CHD.

Pilot study of chronic maternal hyperoxygenation and effect on aortic and mitral valve annular dimensions in fetuses with left heart hypoplasia.

Acute maternal hyperoxygenation (AMH) results in increased fetal left heart blood flow. Our aim was to perform a pilot study to determine the safety, feasibility and direction and magnitude of effect of chronic maternal hyperoxygenation (CMH) on mitral and aortic valve annular dimensions in fetuses with left heart hypoplasia (LHH) after CMH.