Prenatal Diagnosis of a Ductal-Dependent Branch Pulmonary Artery: Extra Vessels in the 3-Vessel and Trachea View.

Obtaining a three-vessel (3V) and three-vessel and trachea (3VT) view from the fetal upper mediastinum is now considered to be part of standard imaging protocol for routine obstetric cardiac screening examinations. We report two fetal cases of an anomalous pulmonary artery origin, utilizing the standard 3V and 3VT views. Further imaging led to a rare diagnosis of bilateral ductus arteriosus with discontinuous branch pulmonary arteries in the absence of any other congenital heart defect. We briefly discuss the imaging features, differential diagnoses, and management of this rare entity.

Determinants of Length of Stay after Neonatal Cardiac Surgery Using Path Analysis.

After neonatal cardiac surgery, families, and the health care team strive for exclusive oral feedings before hospital discharge. With the hypothesis that exclusive oral feedings would reduce the length of stay (LOS), a multidimensional path analysis was used to examine a cross-section of 280 neonates from 2009 to 2013. Buttigieg, Abela, and Pace's theoretical framework of structural and process-related determinants of LOS was modeled with hypothesis-driven correlation and directionality. The recursive path model had a good global and local fit with outcome variances of 26% for exclusive oral feeding and LOS. In the full cohort and model groups (single and biventricular), when controlling for covariances: sepsis, birth distance, necrotizing enterocolitis, genetic differences, specialty consults, the age at which neonatal cardiac surgery occurred (ß = .23, p ≤ .001) and the duration of postoperative intubation (ß = .47, p ≤ .001) more significantly influenced the LOS than intermediate mediation of exclusive oral feedings at discharge.

Pediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease.

In order to evaluate physicians' willingness to seek legal action to mandate surgery when parents refuse surgery for various congenital heart lesions, we surveyed pediatric cardiologists and cardiovascular surgeons at 4 children's hospitals. We asked whether physicians would support parental refusal of surgery for specific heart defects and, if not, whether they would seek legal action to mandate surgery. We then analyzed associations between physicians' willingness to mandate surgery and national operative mortality rates for each lesion. We surveyed 126 cardiologists and 9 cardiac surgeons at four tertiary referral centers. Overall response rate was 77%. Greater than 70% of physicians would seek legal action and mandate surgery for the following lesions: ventricular septal defect, coarctation of the aorta, complete atrioventricular canal, transposition of the great arteries, tetralogy of Fallot, and unobstructed total anomalous pulmonary venous return. Surgery for all of these lesions has reported mortality rates of < 5%. Physicians were less likely to seek legal action when parents refused surgery for Shone complex, any single ventricle lesion, or any congenital heart disease accompanied by Trisomy 13 or Trisomy 18. Among experts in pediatric cardiology, there is widespread agreement about the appropriate response to parental refusal of surgery for most congenital heart lesions, and these lesions tended to be heart defects with lower surgical mortality rates. Lesions for which there was greater consensus among experts were those with the best outcomes. There was less consensus for lesions with higher mortality rates. Such surveys, revealing disagreement among expert professionals, can provide an operational definition of the current professional "gray zone" in which parental preferences should determine treatment.

Improving Wait Time for Patients in a Pediatric Echocardiography Laboratory - a Quality Improvement Project.

BACKGROUND: Prolonged wait times for echocardiograms in the outpatient pediatric cardiology clinic led to patient and provider dissatisfaction at our institution. The aims of this project were to measure our baseline performance with regard to echocardiogram wait time (EWT), to implement a formal quality improvement (QI) program to improve EWT, and to measure the impact of QI on EWT. METHODS: A QI team was formed comprising of cardiologists (A.P., T.S.), sonographer (A.W.), and QI mentor (J.M.). EWT was defined as time in minutes from initiation of the order in the electronic medical record to start of the echocardiogram. Goal EWT was set as ≤ 20 minutes for 90% patients. Flowcharts were created after process observation to identify sources of potential delay contributing to EWT. QI methodology such as driver diagrams were utilized to identify interventions, which were then implemented and studied as Plan-Do-Study-Act cycles. RESULTS: Sequential interventions included early start time, huddles involving clinic and echo laboratory staff, patient tracking system, and repurposing of a clinic room for echo. EWT was tracked for 840 patients. Mean EWT was 22.5 ± 17.5 minutes at baseline and decreased to 15.3 ± 7.8 minutes postintervention (P < 0.001). Postintervention, 81% of the patients waited < 20 minutes for their echo, and 98% patients waited < 30 minutes, compared with baseline numbers of 62% and 76%, respectively (P < 0.001). CONCLUSIONS: We were able to utilize QI methodology to derive interventions and track changes, resulting in quantifiable improvement in EWT in a busy pediatric echo laboratory.

Thoracic ganglioneuromas resulting in nonimmune hydrops fetalis.

Introduction Most often, ganglioneuromas affect older pediatric and adult patients. They are typically slow growing tumors that remain clinically silent until they become large enough to cause symptoms by compression of adjacent structures. Case We report a case of a 22-year-old Hispanic gravida 2 para 1 female patient who was found to have massive hydrops fetalis at 20 completed gestational weeks. Fetal echocardiography revealed a narrowed distal ductal arch and proximal descending aorta. Cesarean delivery was undertaken at 29 completed gestational weeks for refractory labor and nonreassuring fetal status. The neonate expired at 47 minutes of life despite aggressive resuscitation. At autopsy, multiple thoracic masses were found adjacent to a compressed proximal descending aorta. Histological and immunohistochemical analysis confirmed the diagnosis of a ganglioneuroma, a rare type of neural crest tumor. Discussion A variety of intrathoracic masses have previously been reported to cause hydrops fetalis including teratomas, fibrosarcomas, and lymphangiomas. To our knowledge, this case is the first description of hydrops fetalis caused by ganglioneuromas. We propose that multiple thoracic ganglioneuromas led to biventricular distal outflow tract obstruction and hydrops fetalis.

Acute and chronic fetal anemia as a result of fetomaternal hemorrhage.

Introduction. Fetomaternal hemorrhage represents a transfer of fetal blood to the maternal circulation. Although many etiologies have been described, most causes of fetomaternal hemorrhage remain unidentified. The differentiation between acute and chronic fetomaternal hemorrhage may be accomplished antenatally and may influence perinatal management. Case. A 36-year-old gravida 6 para 3 presented at 37 and 5/7 completed gestational weeks with ultrasound findings suggestive of chronic fetal anemia such as right ventricular enlargement, diminished cerebral vascular resistance, and elevated middle cerebral artery end-diastolic velocity. On the other hand, signs of acute fetal decompensation such as deterioration of the fetal heart tracing, diminished biophysical score, decreased cord pH, and increased cord base deficit were noted. Following delivery, the neonate's initial hemoglobin was 4.0 g/dL and the maternal KB ratio was 0.015 indicative of a significant fetomaternal hemorrhage. Discussion. One should consider FMH as part of the differential diagnosis for fetal or immediate neonatal anemia. We describe a unique case of FMH that demonstrated both acute and chronic clinical features. It is our hope that this case will assist practitioners in differentiating acute FMH that may require emergent delivery from chronic FMH which may be able to be expectantly managed.

The feasibility and acceptability of neuromuscular electrical stimulation to improve exercise performance in patients with advanced cancer: a pilot study.

BACKGROUND: To determine the feasibility and acceptability of lower limb neuromuscular electrical stimulation (NMES) as a home-based exercise therapy in patients with cancer who could not attend hospital-based exercise training. METHODS: A single-arm prospective pilot study of NMES, applied daily to both quadriceps muscles for six weeks. Participants were recruited from patients referred to a hospital-based multi-disciplinary supportive care team specializing in treatment of patients with nutritional depletion and functional decline. RESULTS: Of the 15 participants who underwent baseline testing, 10 (67%) completed the study and only one (7%) withdrew because of discomfort due to NMES treatment. 7/10 (70%) of participants used NMES at least three times a week for the duration of the study. Use of NMES did not lead to significant improvements in physical performance tests. CONCLUSIONS: NMES is a feasible and acceptable intervention for home use in patients with cancer, poor performance status and metastatic disease. However, whether NMES is an effective strategy to stabilize or improve physical performance in such patients is not proven.

Weight changes correlate with alterations in subjective physical function in advanced cancer patients referred to a specialized nutrition and rehabilitation team.

PURPOSE: The aim of this study is to assess whether short-term weight gain correlates with improvements in subjective markers of quality of life and physical function in patients referred to a clinic for management of cancer cachexia. METHODS: A retrospective review of the records of 306 patients referred to a specialized multi-disciplinary supportive care team with particular interest in treating cancer cachexia. Weight changes between each of the first three clinic visits, were correlated with the corresponding changes in patient-rated performance status, perceived strength and quality of life. In a second cohort of 56 patients, the correlation between perceived strength and quality of life was re-tested using a more detailed quality of life tool. RESULTS: Even over short time intervals positive correlations were observed for weight change vs. change in patient-rated performance status (Rs > 0.15, P < 0.05), and for changes in perceived strength vs. quality of life (Rs > 0.33, P < 0.001). The correlation between changes in patient-rated strength and quality of life was consistent across all subgroups studied and was reproducible when using a different, validated, quality of life tool (FAACT) in a second independent patient cohort. CONCLUSIONS: Weight gains are associated with subjective improvements in physical functioning, and changes in perceived physical strength are consistently correlated with quality of life.

Truncus arteriosus: diagnostic accuracy, outcomes, and impact of prenatal diagnosis.

Limited data exist on the impact of prenatal diagnosis and outcomes of fetal truncus arteriosus (TA). We sought to assess prenatal diagnostic accuracy and prenatal outcomes in fetuses with TA and compare postnatal outcomes in neonates with prenatally and postnatally diagnosed TA. Records were reviewed for patients diagnosed with TA in utero or at

Setting properties of four acrylic and two calcium-phosphate cements used in vertebroplasty.

STUDY DESIGN: Experimental study conducted in the laboratory with six different bone cements. OBJECTIVES: To isolate the thermal properties of conventional and emerging bone cements used in vertebroplasty and to characterize their setting behavior. SUMMARY OF BACKGROUND DATA: The heat released during setting has been linked to the desirable effects of pain relief and tumor destruction and to the undesirable effect of thermal necrosis of surrounding tissue. However, there are currently no studies that disconnect the exothermic reaction of the cements from the media in which they occur. Before the combined thermal effect is examined, it is important to understand the setting properties of cements alone. METHODS: Thirty independent experiments were conducted with four PMMA cements (Cranioplastic, Vertebroplastic, Palacos LV-40, Antibiotic Simplex) and two calcium-phosphate cements (chronOS Inject and Biopex) in accordance with ASTM standard F 451-99a. A thermocouple was placed in the center of the cement mass, and the temperature-versus-time measurements were recorded. RESULTS: The calcium-phosphate cements took over half an hour to reach their maximum temperature, which was only 3-4 degrees C higher than the ambient temperature. The temperature increase for the acrylic cements was between 16 and 23 degrees C, and it took about 15 minutes to reach the maximum temperature. The variation within the groups was also important. CONCLUSION: The exothermic reaction of calcium-phosphate cements appears to be insignificant. Although the acrylic cements release considerably greater heat in a much shorter time period, it does not appear that their temperature is sufficiently high to cause extensive thermal injury. However, variations within each group must be considered along with the intended use when deciding on the cement to be used.