Interstudy variability in cardiac magnetic resonance imaging measurements of ventricular volume, mass, and ejection fraction in repaired tetralogy of Fallot: a prospective observational study.

PURPOSE: To assess the interstudy variability of cardiac magnetic resonance imaging (CMR) parameters of ventricular size and function in repaired tetralogy of Fallot (TOF). MATERIALS AND METHODS: Patients with TOF (n = 30, median age 23.5 years, 43% male) were enrolled prospectively. Each patient underwent two consecutive CMR examinations on the same day. Each examination was analyzed for ventricular size and function by two observers and multiple comparisons were made with assessment of agreement using Bland-Altman analysis and intraclass correlation coefficients (ICC). RESULTS: Agreement for most measures of ventricular size and function was high when a single observer analyzed both studies. Agreement was worse when different observers analyzed sequential studies. This effect was most prominent on measurements of right ventricular (RV) mass and there was slight improvement when mass was measured during systole. Aside from ventricular mass, agreement was similar for RV and left ventricular (LV) parameters. CONCLUSION: CMR measures of ventricular size and function have acceptable repeatability across serial examinations in patients with repaired TOF. Measurements of RV mass are subject to higher variability. For most parameters, agreement limits are wider when measurements are performed by multiple operators. These results will aid in the interpretation of study-to-study variations in the follow-up of individual patients and in designing future clinical trials.

Congenital arteriovenous malformation between the subclavian artery and superior vena cava presenting in neonatal heart failure.

Congenital arteriovenous malformations are a well-described cause of neonatal heart failure. Fistulous connections are typically intrahepatic or intracranial. We present a case of a neonate with an intrathoracic arteriovenous malformation between the subclavian artery and superior vena cava resulting in florid neonatal heart failure. This unusual fistulous connection has only rarely been reported in the literature, and in those reports, it has not resulted in neonatal heart failure.

Oxygen supplementation is helpful for the echocardiographic detection of anomalous left coronary artery from the pulmonary artery.

BACKGROUND: The echocardiographic diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA) can be challenging. The aim of this study was to assess the hypothesis that diagnosis can be enhanced by using supplemental oxygen, which decreases pulmonary vascular resistance and increases retrograde flow from the coronary artery into the pulmonary artery. METHODS: Demographic, echocardiographic, and cardiac catheterization data were reviewed in patients presenting with ALCAPA from 1999 to 2007. RESULTS: Twenty-one patients (seven male; median age, 5 months) presented with ALCAPA. Nine underwent imaging with oxygen. Two of these nine (22%) had previous standard echocardiographic studies that missed the diagnosis. Cardiac catheterization was required for diagnosis of ALCAPA in 42% of patients who underwent standard echocardiography compared with 11% of patients who received supplemental oxygen in addition to standard echocardiography. The administration of oxygen caused no significant change in heart rate or cardiorespiratory support. CONCLUSION: Transient oxygen administration is useful in the noninvasive diagnosis of ALCAPA.

Long-term outcomes with ambrisentan monotherapy in pulmonary arterial hypertension.

BACKGROUND: This study evaluated long-term outcomes in patients with pulmonary arterial hypertension (PAH) undergoing treatment with ambrisentan monotherapy, a selective oral endothelin-1 receptor antagonist. METHODS AND RESULTS: Patients who participated in the Ambrisentan in Pulmonary Arterial Hypertension: A Phase 3, Randomized, Double-Blind, Placebo-Controlled Multicenter Efficacy Study (ARIES-1) clinical trial and extension phase at our institution were included. Cardiac catheterization, 6-minute walk distance (6MWD), and cardiac magnetic resonance (MRI) data were retrospectively reviewed. Twelve patients with PAH (11 idiopathic, 1 fenfluramine) had follow-up from 3 to 5.5 years from the initiation of ARIES-1. Patients received ambrisentan therapy throughout the study period and were on ambrisentan monotherapy for the first 2 years. At year 1, improvements in median mean pulmonary arterial pressure (PA), cardiac output, and pulmonary vascular resistance (PVR) were seen (P = .02, P = .03, P < .01), and the improvement in PVR persisted at 2 years. 6MWD also improved significantly between baseline (350 m) and 1 and 2 years (397 m, P < .01 and 393 m, P = .01). Cardiac MRI results were more varied, with an increase in RV ejection fraction from 29% at baseline to 46% at 2 years (P = .02), but other MRI variables did not improve. CONCLUSIONS: Ambrisentan monotherapy led to improvements in catheterization, 6MWD, and RV ejection fraction, and shows promise as a long-term treatment for pulmonary arterial hypertension.

Patent ductus arteriosus in micropreemies and full-term infants: the relative merits of surgical ligation versus indomethacin treatment.

BACKGROUND/PURPOSE: Treatment of ductus arteriosus is controversial. The merits and timing of ductus ligation versus indomethacin treatment were compared. METHODS: Study parameters for infants with PDA (June 1988 through March 2001) included age, weight, physical findings, echocardiograph, success/complications of treatment, and length of stay (LOS). Statistical analysis was by descriptive univariate analysis. RESULTS: Two hundred twelve infants were included. Median gestational age was 26 weeks (range, 22-38); weight, 836 g (447 to 2,863). Findings included murmur (94%), congestion (20%), cardiomegaly (15%), bounding pulses (6%), hyperdynamic precordium (4%), and CHF (2%). Echocardiographic measurements were left atrial diameter, 0.92 cm; posterior wall thickness, 0.26 cm; PDA diameter, 2.5 mm; septal thickness, 0.27 cm; aortic root diameter, 0.64 cm; ejection fraction, 39%; left ventricular internal diameter-diastole, 1.3 cm; left ventricular internal diameter-systole, 0.82 cm; right ventricular internal diameter-diastole, 0.51 cm. No measurement, except PDA diameter, was predictive of medical failure or need for reoperation. However, weight less than 1,000 g was highly predictive of medical failure. Additional cardiac anomalies included ASD (69%), VSD (3%), and aortic coarctation (1%). One hundred sixty-seven babies (79%) received indomethacin, closing the PDA in 88 cases (53%, recurrence = 47%). Indomethacin complications (73%) included thrombocytopenia (36%), BUN increase (31%), sepsis (30%), oliguria (25%), hyponatremia (25%), IVH (16%), pulmonary interstitial emphysema (11%), NEC (8%), intestinal perforation (4%), and bleeding (3%). Seventy-seven babies (36%) required operations (92% in the NICU) performed by pediatric surgeons. Ligation criteria included hypoxia, hypercapnia, decreasing compliance, CHF, and contraindications/failure of indomethacin. Complications included pneumothorax (4%), IVH (4%), bleeding (4%), NEC (1%), and wound infection (1%). LOS averaged 82 days. CONCLUSIONS: Although indomethacin therapy is a reasonable treatment alternative, it is associated with significant complications. Ductus ligation may be preferable, especially in very low birth weight babies, because it is associated with low morbidity and almost certain degree of success.

Laparoscopic appendectomy: An unnecessary and expensive procedure in children?

BACKGROUND/PURPOSE: Laparoscopic Appendectomy (LA) is a safe procedure in adults resulting in shorter hospitalization and sooner return to activity. The relative merits of LA and open appendectomy (OA) are evaluated in this first prospective and randomized study in children. METHODS: A total of 129 children with appendicitis were included. Forty-three boys and 45 girls, age 1 to 16 years, were enrolled. Randomization was determined by sealed assignment card. OA utilized a 3- to 4-cm right lower quadrant, muscle-splitting incision. Wounds were closed without drains. Antibiotics, when used, consisted of gentamycin, clindamycin, and ampicillin. LA was performed by experienced surgeons utilizing a 3-trocar technique with reusable instruments. Twenty-one children (24%) were perforated. Patients were discharged as soon as they were taking a diet and afebrile. Statistical comparisons were by Fisher's Exact and Wilcoxon rank-sum tests. RESULTS: There were no differences in postoperative analgesia, resumption of oral intake, length of hospitalization, return to normal activities, or morbidity. Laparoscopic appendectomy was associated with longer operating times and increased cost. CONCLUSIONS: Laparoscopic appendectomy in children is not associated with the same advantages reported in adults. LA is a more expensive alternative and offers no advantages related to pain relief, length of stay, return to normal activities, or morbidity.