Surgical Management Of Neonates Research Articles

Optimising the surgical management of neonates with hypoplastic left heart syndrome

Optimising the surgical management of neonates with hypoplastic left heart syndrome

Surgical management of symptomatic neonates with Ebstein's anomaly: Choice of operation

Objective: Symptomatic neonates with Ebstein's anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. Methods: A total of 32 consecutive symptomatic neonates with Ebstein's anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients' weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two- ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein's anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes' palliation and 2 Blalock-Taussig shunts. Six recent patients with Ebstein's anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. Results: Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein's anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). Conclusions: Surgical management of neonates with Ebstein's anomaly remains challenging. For neonates with Ebstein's anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two- ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.

Surgical management of symptomatic neonates with Ebstein's anomaly: choice of operation.

Symptomatic neonates with Ebstein's anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. A total of 32 consecutive symptomatic neonates with Ebstein's anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients' weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein's anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes' palliation and 2 Blalock-Taussig shunts. Six recent patients with Ebstein's anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein's anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). Surgical management of neonates with Ebstein's anomaly remains challenging. For neonates with Ebstein's anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.

Clinical Issues and Outcomes in Adults Following Repair of Tetralogy of Fallot

Advances in diagnosis, perioperative and surgical management of neonates born with Tetralogy of Fallot (TOF) have meant that affected individuals can now expect to survive to adulthood. However, this success is counterbalanced by the development of late morbidity in adulthood affecting survival and functional capacity. This review article addresses some of the major sequelae of TOF repair and discusses the contemporary approaches to reduce morbidity and mortality in this population.

Hybrid procedure as an alternative to surgical palliation of high-risk infants with hypoplastic left heart syndrome and its variants

Despite improvements in the surgical management of neonates with the hypoplastic left heart syndrome and its variants, there is a subset of children who have a high predicted mortality. We have analyzed and report our results with the hybrid approach in the management of these high-risk patients. Children identified by our selection criteria underwent the initial hybrid procedure performed in the catheterization laboratory. This consists of surgical banding of the branch pulmonary arteries and stenting of the arterial duct. This was followed 3 to 6 months later by the combined stage I and II Norwood operation or biventricular repair. Between December 2005 and May 2008, 21 neonates were selected for the hybrid procedure, of whom 16 had typical hypoplastic left heart syndrome. The remaining 5 neonates had severe aortic stenosis with borderline left ventricular dimensions or poor left ventricular function at presentation. Four neonates had a virtually intact interatrial septum and required urgent intervention immediately after birth. There were 6 deaths as inpatients or up to 30 days after the procedure and 1 interstage death before the combined stage I and II operation. Thus far, of 12 babies who have undergone the next operation, 9 have had the combined stage I and II operation and 3 have had a biventricular repair. Two of these 12 babies have died after the combined stage I and II operation. A hybrid approach may be a valuable alternative to the management of high-risk neonates with hypoplastic left heart syndrome and its variants.

Surgical management of neonates with congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is one of the most challenging and complex pediatric abnormalities to manage, both medically and surgically. The care of these neonates has seen significant evolution, from previous aggressive ventilation and emergent operation to current permissive hypercapnea, physiologic stabilization, and elective surgical repair, all in less than two decades. These changes have led to many centers reporting survival rates near 80%, a dramatic improvement from the 50% survival reported in the 1970s. This review covers the current principles guiding the surgical management of CDH in the neonate, including preoperative stabilization, operative timing, extracorporeal membrane oxygenation, surgical approach, and management of recurrence. Although many clinical challenges remain, multi-institutional collaboration and ongoing research efforts will hopefully improve the clinical care of these patients.

Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS) occurs in 5% of patients with TOF. TOF-APVS includes an anterior malalignment ventricular septal defect (VSD) similar to TOF with pulmonary stenosis. The right ventricle is hypertrophied and may contain obstructing muscle bands in addition to infundibular narrowing. The pulmonary annulus is mildly to moderately hypoplastic with vestigial nubbins of nonfunctional myxomatous tissue rather than developed valve leaflets. The size of the main pulmonary artery ranges from normal to aneurysmal depending on the severity of the anomaly, whereas the branch pulmonary arteries are markedly aneurysmal, often two to three times their normal diameters. The hilar portions of the pulmonary arteries may also be dilated. The segmental branches are of normal size and appear as multiple orifices arising from the dilated portion of the artery when viewed from inside the vessel. A patent ductus arteriosus is never present in TOF-APVS. The combination of a VSD, pulmonary insufficiency, and a patent ductus arteriosus would potentially produce physiologic aortic insufficiency: left ventricular output could flow retrograde through the ductus, into the right ventricle, across theVSD,andbackintotheleftventricle.Ithasbeenproposed that the absence of a pulmonary valve influences premature closure of the ductus, or the fetus would not survive. The highly pulsatile flow through the pulmonary arteries may result in the aneurysmal dilation. 1,2 Patients with TOF-APVS can be divided into two groups. The first group presents in the neonatal period with respiratory distress, attributed to compression of the tracheobronchial tree by the aneurysmal pulmonary arteries. These patients require early surgery to avoid morbidity and death from respiratory distress and heart failure. The second group of patients is less symptomatic in infancy and may be identified later in life with symptoms of congestive heart failure, or classic TOF cyanotic spells. These patients can be managed similarly to other patients with TOF and undergo elective repair. There are two major debated issues regarding the surgical management of neonates with TOF-APVS: management of the aneurysmal pulmonary arteries, and placement of a pulmonary valve in the right ventricular outflow tract. Multiple operations have been proposed to address these issues. 3-10 In the operation we describe below, we remove the maximal amount of abnormal pulmonary arterial tissue and place a right ventricular to pulmonary artery homograft conduit. Complete excision of the main and branch pulmonary arteriesremovesalmostallaneurysmaltissue,therebyminimizing any chance of persistent tracheobronchial compression. Use of a homograft places a valve in the pulmonary outflow tract, which simplifies the postoperative management of pulmonary hypertension.

Outcomes of 829 neonates with complete transposition of the great arteries 12-17 years after repair.

Between 1985 and 1989, the surgical management of neonates with complete transposition (TGA) underwent a transition from atrial to arterial repair. We sought to examine the intermediate outcomes and their associated risk factors in neonates repaired during the era of transition. Twenty-four institutions entered 829 neonates age less than 15 days in a prospective study. Diagnosis was simple TGA (n=631), TGA with ventricular septal defect (VSD) (n=167), TGA with VSD and pulmonary stenosis (TGA/VSD/PS) (n=30), or TGA with PS (n=1). Repair was by arterial switch (n=516), atrial repair (Senning=175, Mustard=110) or Rastelli (n=28). Time-related events were analysed by parametric hazard function modeling and incremental risk factors for mortality, re-intervention, and late functional assessment were sought. Survival estimates at 6 months, 5, 10, and 15 years are 85, 83, 83, and 81%, respectively. The hazard function for death after repair has two phases: an early rapidly declining phase and an ongoing constant one. Constant phase mortality is less likely after the arterial switch operation and in children with simple TGA. During follow up, at least one re-intervention was required in 167 children (pacemaker, n=35; percutaneous intervention, n=32; baffle re-intervention, n=27; re-operation, n=125). Freedom from re-intervention at 6 months, 5, 10 and 15 years is 93, 82, 77, and 76%, respectively. Of survivors, 87% have been followed up to the last 3 years, including an assessment of functional ability of 562 children (83%). Functional class 15 years after repair is class I in 76%, II in 22%, III in 2%. The proportion in functional class I decreased over time. Psychosocial deficits, especially learning disorders are prevalent. Survival 15 years after TGA repair is good with most children functioning well, and results are best after an arterial switch operation. There is an ongoing risk of death that is less after the arterial switch operation. With the exception of Rastelli patients, the likelihood of survivors needing re-intervention after 5 years is low. There is need for improved neurodevelopmental outcomes.

The clinical significance of thrombocytopenia in neonates with necrotizing enterocolitis

Purpose: The aim of this study was to evaluate the clinical significance of low platelet count in the surgical management of neonates with necrotizing enterocolitis (NEC). Methods: The clinical course of 58 consecutive neonates with advanced NEC (Bell's stages II or III) treated in the authors' hospital between 1995 and 1998 was reviewed. NEC in neonates who required operation was classified as isolated, multifocal, or pan-intestinal. Severe thrombocytopenia was defined as platelet count less than 100 × 109/L. Rapid fall in platelet count was defined as fall greater than 150 × 109/L within 24 hours to a level less than 100 × 109/L. Results: Median birth weight was 1,564 g (range, 550 to 4,270) and gestational age was 31 weeks (range 23 to 41). Twenty-two neonates (38%) were below 1,000 g. Age at the onset of the disease was 13 days (range, 1 to 62). NEC was treated medically in 7 neonates (12%). Indications for operation included pneumoperitoneum in 23 neonates (45%), clinical deterioration in 19 (37%), and intestinal obstruction in 9 (18%). The nadir platelet count (lowest level during the course of disease) was lower in patients with stage III disease than in patients with stage II disease (P <.05). The greater the extent of the disease, the lower the platelet count (P =.012). The nadir platelet count was lower in infants who died than in survivors (P <.05). None of the patients with platelet count greater than 100 × 109/L died. In predicting intestinal gangrene, severe thrombocytopenia has a sensitivity of 69%, specificity of 60%, and positive predictive value of 89%; rapid fall in platelet count has a sensitivity of 32%, specificity of 89%, and positive predictive value of 92%. Conclusions: (1) A platelet count less than 100 × 109/L or a rapid fall in platelet count represent poor prognostic factors. (2) Monitoring the platelet count during the course of NEC is useful; however, it cannot be used in isolation to predict the extent of the disease or survival rate. J Pediatr Surg 36:799-803. Copyright © 2001 by W.B. Saunders Company.

Surgical Management of Neonates with Complex Preductal Aortic Coarctation

Neonates having repair of aortic coarctation commonly have associated ventricular septal defect and patent ductus arteriosus. Prostaglandin E1 is used to dilate the ductus and improve the patient's preoperative condition. An operative technique that maintains ductal patency until the final stages of anastomosis is presented. We believe it has contributed to our present improved results.