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Ethical Issues in the Care of Children with Necrotizing Enterocolitis
Published in David J. Hackam, Necrotizing Enterocolitis, 2021
Margaret R. Moon, Renee D. Boss
Ultimately, parents and clinicians may hold different values about what chance of a good outcome is required to trigger surgical intervention. Sometimes families desire surgery for an infant with very serious comorbidities and an acute clinical decompensation concerning for NEC totalis. These families may have already pursued medical or surgical interventions for their infant despite grave predictions about, for instance, hypoplastic left heart syndrome. Such families can be overly hopeful and require skillful counseling. Other families may refuse surgery even as clinicians believe it offers the infant meaningful recovery. Kunz et al. describe a case of parent–clinician conflict about the value of surgery for a medically fragile infant with NEC; the authors highlight a distinction to be made between diagnostic laparotomy versus bowel resection (8). Families and clinicians should consider whether the prognostic information from laparotomy may be of value even when bowel resection may not.
3D Printing
Published in Takahiro Shiota, 3D Echocardiography, 2020
3D echocardiography has also been used for 3D printing of pediatric atrioventricular valves. Mastering the technical skills required to perform successful valve repair in pediatrics is challenging due to the very small and growing structures and the limited opportunity for practice. Therefore, there is a need for valve model–based simulation training in pediatrics and congenital heart disease. Scanlan et al. created 3D printed models of atrioventricular valves entirely dependent on 3D echocardiography datasets9 (Figure 21.6). They included a range of children with normal and congenital heart disease including hypoplastic left heart syndrome and atrioventricular septal defects.
Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Hypoplastic left heart syndrome is characterised by underdevelopment of left heart structures. This is uniformly fatal in the first few weeks of life without intervention. Treatment includes prostaglandin therapy to maintain ductal patency, followed by resuscitation with inotropes, intubation and judicious ventilatory management to minimise pulmonary overcirculation, which is invariably present because of the need to maintain ductal patency in order to maintain systemic perfusion to the descending thoracic aorta via the ductus. Occasionally, subambient oxygen is needed in order to increase pulmonary vascular resistance and avoid pulmonary vascular congestion. Pulmonary overcirculation is manifested by tachypnoea, tachycardia, intercostal retractions and higher than usual oxygen saturations, typically >85%. Ideal oxygen saturations for single ventricle patients should be 75%–85%, which provides enough of a balance between systemic and pulmonary perfusion. Treatment is via the Norwood procedure, which involves aortic arch augmentation with a patch, a side-by-side anastomosis of the main pulmonary artery and the diminutive ascending aorta, to reconstruct a neoaortic outflow from the ventricular mass. Pulmonary blood flow is via a modified Blalock– Taussig shunt (from the innominate artery to the pulmonary artery) or via a Sano shunt (from the right ventricle to the pulmonary artery). Subsequent operations are the Glenn procedure (at 6 months of age) and the Fontan procedure (at approximately 2–3 years of age).
Lactobacillus rhamnosus Sepsis Associated with Probiotic Therapy in a Term Infant with Congenital Heart Disease
Published in Fetal and Pediatric Pathology, 2022
Seda Aydoğan, Dilek Dilli, Ahmet Özyazici, Nesibe Aydin, Hüsniye Şimşek, Utku Arman Orun, Ömer Nuri Aksoy
An eight-day-old male patient was admitted to our NICU with a rapid respiratory rate of 74/min (reference range:20-60/min) and respiratory failure. He was born by vaginal delivery at term with a birth weight of 2500 g. CoA and borderline hypoplastic left heart syndrome (HLHS) was detected by echocardiography. He underwent aortic repair and pulmonary banding operation on the postnatal 24th day. During the NICU stay, the patient needed long-term mechanical ventilation (MV) due to cardiorespiratory failure. Recurrent systemic infections (pneumonia and sepsis caused by Klebsiella pneumonia) developed. We began enteral probiotic supplementation to reduce nutritional intolerance and to prevent NEC as he could not be fed sufficiently on the 10th day of surgery. On the 12th day of probiotic therapy, (Lactobacillus rhamnosus GG 1 × 109 colony-forming unit (CFU), Maflor®), the patient developed fever and nutritional intolerance. Laboratory results included a C- reactive protein of 47.8 mg/L (reference range: <1mg/L), white blood cell count of 13,050 cells/mL (reference range: 5,000-15,000), neutrophil count of 9,220 cells/mL (reference range:1,800-5,400), and a platelet count of 102,000 cells/mL (reference range:15,0000-400,000). During this period, the patient was under treatment with colistin, liposomal amphotericin B and meropenem based on the culture results and NICU flora. Lactobacillus rhamnosus grew in blood culture.
Current evidence for pharmacologic therapy following stage 1 palliation for single ventricle congenital heart disease
Published in Expert Review of Cardiovascular Therapy, 2022
Meredith C. G. Broberg, Ira M Cheifetz, Sarah T Plummer
The survival of patients with congenital heart disease has significantly improved over the past several decades. Hypoplastic left heart syndrome (HLHS) and other single ventricle heart lesions were uniformly fatal 40 years ago. Now, many patients with HLHS undergo staged palliation with survival ranging from 70% to 90% [1, 2]. The palliative approach includes stage 1 surgical palliation in the neonatal period with either the Norwood procedure with a modified Blalock-Taussig shunt (mBTS) or a right ventricle to pulmonary artery conduit (RV-PA conduit); or a hybrid procedure with stenting of the patent ductus arteriosus and bilateral branch pulmonary artery band placement. At approximately 4–6 months of age patients undergo stage 2 palliation, most commonly, with a superior cavopulmonary anastomosis (bidirectional Glenn procedure). This is followed by stage 3 palliation with the Fontan operation at generally 2–4 years of age. The stage 2 superior cavopulmonary anastomosis and stage 3 Fontan procedures have excellent surgical outcomes [3]. Mortality, however, is greatest following stage 1 palliation and within the first interstage period – the time between stage 1 and stage 2 operations [4].
Prenatal diagnosis of aortopulmonary window by foetal echocardiography: ‘U or reversed U sign’
Published in Journal of Obstetrics and Gynaecology, 2019
Başak Kaya, Ali Ekiz, Deniz Kanber Acar, Salim Sezer, Helen Bornaun, Gökhan Yildirim, Halil Aslan
A 26-year-old, primigravid, monochorionic diamniotic pregnant woman was referred to our hospital at 25 weeks of gestation because of suspected congenital heart disease. One foetus had no sign of cardiac and extracardiac anomaly. However, in the second foetus, a small hypo-contractile left ventricle was observed. The mitral valve was dysplastic and a hypoplastic transverse aortic arch with antegrade flow was observed in the three-vessel-trachea view. The three-vessel and three-vessel-trachea views also demonstrated a communication between the hypoplastic ascending aorta and the main pulmonary artery. In short axis view, the presence of an aortopulmonary septal defect with a right-to-left shunt was confirmed (Figure 1 (c,d)). The patient did not accept a foetal chromosome analysis. At 28 weeks of gestation, a caesarean section was performed due to preterm labour, and two male infants who were 930 and 850 grams were delivered. The diagnosis of type-I APW with hypoplastic left heart syndrome was confirmed by postnatal echocardiography. The baby died at the age of 9 days due to prematurity and cardiopulmonary complications.