Fetal Doppler Echocardiographic Assessment Predicts Severe Postnatal Obstruction in Total Anomalous Pulmonary Venous Connection.

BACKGROUND: Obstructed total anomalous pulmonary venous connection (TAPVC) is a form of critical congenital heart disease that usually requires urgent postnatal intervention. Knowing which patients have severe obstruction can aid delivery planning. The authors previously developed a novel quantitative metric of pulmonary venous flow, the pulmonary venous variability index (PVVI). The aim of this study was to test the hypothesis that fetal PVVI and vertical vein Doppler velocities are associated with severe pulmonary vein obstruction postnatally. METHODS: A retrospective cohort study of neonates with prenatally diagnosed TAPVC was performed. Patients who underwent fetal echocardiography at the Children's Hospital of Philadelphia with Doppler interrogation of the vertical vein were included for analysis. Twenty-nine patients met criteria (21 with heterotaxy, 18 with supracardiac TAPVC). The latest gestation fetal echocardiogram was used. Severe pulmonary vein obstruction was defined as preoperative death or urgent surgery or catheter-based intervention (first day of life). Measurements of PVVI, defined as (maximum velocity - minimum velocity)/mean velocity, were made offline. Wilcoxon rank sum models were used to assess the associations of severe obstruction and PVVI and maximum, mean, and minimum velocities. RESULTS: The mean gestational age at the latest fetal echocardiographic examination was 35 weeks (range, 30-39 weeks). Twelve of the 29 patients (41%) met criteria for severe pulmonary vein obstruction. Lower PVVI was associated with greater risk for severe pulmonary venous obstruction (P = .008). The maximum, mean, and minimum velocities in the vertical vein were all significantly associated with severe pulmonary venous obstruction (P = .03, P = .03, and P = .007, respectively). Qualitative assessment of obstruction was not significantly associated with the outcome. Interobserver reliability for all vertical vein Doppler metrics was high (intraclass correlation coefficient > 0.9). CONCLUSIONS: Fetal PVVI and maximum, mean, and minimum velocities are associated with severe postnatal pulmonary vein obstruction in TAPVC. Accurate prediction of obstructed TAPVC could allow safer delivery planning. Further research with larger sample sizes is needed to identify the ideal cutoff values for these Doppler measures.

The Prevalence of Left Ventricular Hypertrophy in Obese Children Varies Depending on the Method Utilized to Determine Left Ventricular Mass.

Obesity and left ventricular hypertrophy (LVH) have been identified as independent risk factors for cardiovascular events. The definition of LVH depends on the geometric algorithm used to calculate LV mass (LVM) by echocardiography and the method used to normalize LVM for body size. This study evaluates the effect of these methods on the prevalence of LVH in obese children. LVM for 109 obese and 109 age-matched non-obese children was calculated using M-mode or two-dimensional echocardiography (2DE). LVM was then normalized to height 2.7 as indexed LVM (LVMI), to body surface area (BSA), height, and lean body mass (LBM) as LVM Z-scores. LVH was defined as LVMI >95th ‰ using age-specific normal reference values or LVM Z-scores ≥2. The prevalence of LVH by LVMI and LVM Z-scores was compared. There was a correlation between LVM determined by M-mode and by 2DE (R (2) = 0.91), although M-mode LVM was greater than 2DE LVM. However, the difference between these values was greater in obese children than in non-obese children. Based on the method of normalization, the prevalence of LVH among obese children was 64 % using LVMI, 15 % using LVM Z-scores for height, 8 % using LVM Z-scores for BSA and 1 % using LVM Z-scores for LBM. Height-based normalization correlates with obesity and hypertension. The methods used to measure and normalize LVM have a profound influence on the diagnosis of LVH in obese children. Further study is needed to determine which method identifies children at risk for cardiovascular morbidity and mortality.

A neonate with critical congenital heart disease.

Critical congenital heart disease (CCHD) is defined as a ductal-dependent congenital heart defect requiring surgical or percutaneous intervention via cardiac catheterization before 1 year of age. Most cases of CCHD can be diagnosed with prenatal ultrasound or fetal echocardiogram. If not prenatally diagnosed, CCHD can be stable in the newborn nursery due to persistent ductal patency, and the patient may only be diagnosed after ductal closure and development of cardiac symptoms at home. In this case, a 6-day-old female presented to the emergency department (ED) floppy with agonal respirations, poor capillary refill, and absent femoral pulses. On the day of presentation, the patient became increasingly fussy, refused feeding, and began to gasp. The patient was transported to the ED for evaluation, where a bedside echocardiogram revealed interrupted aortic arch (IAA), ventricular septal defect, minimal flow through a thread-like ductus arteriosus, and severely depressed cardiac function. IAA is very rare, with an incidence of three cases per 1 million live births. Patients require neonatal supportive care, continuous prostaglandin E1 infusion, and urgent referral for neonatal surgical repair in the first days to weeks of life. To reduce the volume of undiagnosed CCHD in the immediate newborn period, the U.S. Department of Health and Human Services Secretary's Advisory Committee on Heritable Diseases in Newborns and Children (SACHDNC) recommended that CCHD screening via pulse oximetry be added to the recommended uniform screening panel. A positive screen results in an immediate referral for an echocardiogram. Fetal diagnosis, newborn screening, and/or careful clinical examination may have resulted in detection of IAA in our patient prior to ductal closure.

Perceptions of an implantable cardioverter-defibrillator: A qualitative study of families with a history of sudden life-threatening cardiac events and recommendations to improve care.

OBJECTIVE: To identify major concerns associated with implantable cardioverter-defibrillators (ICDs) and to provide recommendations to adult and pediatric physicians involved in the care of patients with ICDs. BACKGROUND: Cardiac ion channelopathies are a well-recognized cause of sudden cardiac death in infants, children, adolescents, and young adults. ICDs are effective in preventing sudden death from fatal arrhythmias in patients with known cardiac channelopathies. There is a paucity of research on the effect of ICDs on quality of life in patients with cardiac channelopathy diagnoses, especially young patients. METHODS: A qualitative study interviewing patients and families affected by inherited arrhythmias was conducted. Fifty participants with personal or family histories of cardiac events or sudden death were interviewed individually or in focus groups by clinical psychologists. All interviews were transcribed verbatim and then analyzed and coded based on current qualitative research theory to identify themes related to the research question. Twenty-four participants discussed ICDs in their interviews. RESULTS: Participants reported concerns about ICDs, and these concerns were categorized into six themes: (1) comprehension and physician-patient communication; (2) anxiety; (3) restrictions and fallacies; (4) complications; (5) utility; and (6) alternative therapy. Participants noted communication breakdowns between providers and their colleagues, and between providers and their patients. Participants and their families experienced many different forms of anxiety, including worry about the aesthetics of the ICDs and fears of being shocked. Multiple restrictions, fallacies, and complications were also cited. CONCLUSION: Interview themes were used to formulate recommendations for counseling and educating patients with ICDs.