Atypically located cardiac haemangioma of the mitral valve.

We report an adolescent with a benign cardiac haemangioma with attachments exclusively to the anterior leaflet of the mitral valve. On the basis of our review of the literature, this study has not been reported previously.

Giant aneurysm of the left anterior descending coronary artery in a pediatric patient with Behcet's disease.

Behcet's disease is a rare autoimmune vasculitis characterized by oral aphthosis, genital ulcers, and ocular and cutaneous lesions. Vascular involvement usually affects the veins more commonly than the arteries, and coronary arterial involvement is extremely rare. We report an adolescent with Behcet's disease who developed a large pseudoaneurysm of the left anterior descending coronary artery requiring a coronary arterial bypass graft.

Successful kidney transplantation from a donation after cardiac death donor with acute renal failure and bowel infarction using extracorporeal support.

As a result of the ever widening disparity between organ supply and demand, a resurgence of interest has occurred in kidney recovery from donation after cardiac death (DCD) donors. New techniques of in situ extracorporeal support offer the potential to reduce warm ischemic injury and optimize donor management prior to organ recovery. In addition, preliminary outcomes using kidneys from selected deceased donors with rising serum creatinine levels have been promising. However, contraindications to successful organ donation and transplantation may include the presence of abdominal compartment syndrome, generalized bowel infarction, refractory shock with profound metabolic and lactic acidosis, and acute anuric renal failure, particularly in the setting of DCD. We report herein the successful recovery and transplantation of kidneys from an unstable donor with the above constellation of conditions in the setting of extracorporeal support after declaration of death by asystole.

ECMO and congenital heart disease.

Although initially designed for respiratory failure, venoarterial extracorporeal membrane oxygenation (VA ECMO) has become a mainstay of therapy in the treatment of patients with congenital heart disease, providing preoperative and postoperative support for infants with temporary impairment of myocardial function. Postoperative support for pulmonary hypertension has allowed dramatic improvements in lesions such as total anomalous pulmonary venous connection. VA ECMO has also proven to be an acceptable bridge to cardiac transplantation in patients too small for currently available ventricular assist devices. Intraoperative elective use of ECMO has also provided a surgical environment with some specific advantages over conventional cardiopulmonary bypass for lesions such as long segment tracheal stenosis. The results of "rescue ECMO" or extracorporeal cardiopulmonary resuscitation (ECPR) has shown that many children with congenital heart disease are very salvageable despite sudden decompensation and arrest. These techniques of VA ECMO have evolved and improved over the last two decades, allowing expanded application of this life saving support.

Successful management of adult smoke inhalation with extracorporeal membrane oxygenation.

Pulmonary complications remain one of the leading causes of mortality in patients with burns. We report two cases of adult patients with thermal and inhalation injuries who were placed on extracorporeal membrane oxygenation (ECMO) and survived. Patient 1 was a 42-year-old male who suffered 15% TBSA and a severe inhalation injury requiring intubation upon arrival to the emergency department. Patient 2 was a 24-year-old female in a house fire who received 20%TBSA and was noted to be in respiratory distress and intubated on the scene by the paramedic team. Three days after admission, patient 1 developed severe respiratory failure. He decompensated, despite maximum conventional management, and was placed on ECMO. After 300 hours of ECMO, his pulmonary function had improved, and he was decannulated. Patient 2 also developed severe refractory respiratory failure and was placed on ECMO. She was decannulated 288 hours later. Both patients were discharged home shortly afterwards and have managed well. ECMO should be considered when patients are facing a respiratory death from inhalation injury on conventional mechanical ventilation.

Video-assisted diaphragm plication in children.

BACKGROUND: Diaphragm paralysis after cardiac surgery may be secondary to phrenic nerve injury by ice, electrocautery, or dissection. Although most are asymptomatic, some patients, particularly children, have significant respiratory compromise. Video-assisted plication may offer more rapid improvement and recovery than thoracotomy in pediatric patients. METHODS: We performed five procedures. The diaphragm was elevated, clamped, oversewn, and tacked down into the pleural gutter. The procedure was performed on 2 infants after repair of total anomalous pulmonary venous connection, on 1 child after the Fontan procedure, on 1 child after repair of tetralogy of Fallot, and on 1 child with congenital eventration. Indications included ventilator dependency, post-Fontan protein losing enteropathy with elevated venous pressures and chronic right lower lobe collapse, persistent atelectasis with recurrent pneumonias, and asymptomatic severe eventration. RESULTS: Ventilator-dependent patients were extubated after 2 and 3 days. The remaining patients were immediately extubated. One patient was discharged the day of surgery and 2 were discharged at 1 and 3 days postoperatively. The remaining 2 were discharged on postoperative day 30 and 45 after continued issues with feeding and prematurity. The child with the eventration had rapid expansion and growth of the left lung over the next few weeks with a normal chest radiograph 3 weeks later. The child with recurrent pneumonia reexpanded her left lower lobe and remains free of infection. There were no wound infections, lung or vascular injuries, or complications from the procedure. All the patients had successful flattening of the hemidiaphragm as documented by chest radiograph, with successful lung reexpansion. CONCLUSIONS: Video-assisted plication of paralyzed diaphragms is effective and safe, involves less morbidity, and has quicker recovery times than traditional open techniques.

Elective extracorporeal support for complex tracheal reconstruction in neonates.

BACKGROUND: Congenital obstructive anomalies of the trachea present unique challenges in reconstruction and perioperative airway management. Complications include anastomotic breakdown, leak and granulation formation related to the complexity of the repair, and difficulties with perioperative airway management. We describe our technique of elective intraoperative and postoperative extracorporeal support to improve surgical exposure and postoperative healing. METHODS: We have performed complex tracheal reconstructions in 4 newborns (2.2 to 4.3 kg) for long segment tracheal stenosis and complete tracheal rings, diagnosed with bronchoscopy and computerized tomography. Three of the 4 infants had other significant anomalies including complex congenital heart disease, hydrocephalus, encephalomalacia, left lung agenesis, facial anomalies, vertebral anomalies, and hand and hip anomalies. The repairs were performed through a median sternotomy using an extracorporeal membrane oxygenation circuit for support. Venoarterial support was used for the sliding tracheoplasty reconstruction. Extracorporeal membrane oxygenation was converted to venovenous for postoperative "airway rest." After diuresis, the lungs were reexpanded and the 4 patients were ventilated and removed from extracorporeal membrane oxygenation at 4, 5, 8, and 9 days postoperatively. Bronchoscopy was performed to evaluate the airway. RESULTS: All patients had excellent healing of the trachea without granulation tissue. There were no complications of extracorporeal membrane oxygenation support or bleeding issues. All 4 patients survived the surgery and immediate postoperative period with 2 late deaths. The child with congenital heart disease expired after 8 weeks after having hepatorenal failure develop. The child with Goldenhar's syndrome and a single left lung died after 5 months in the hospital. The other 2 patients survived. Two of the infants required late tracheostomy for facial and laryngeal anomalies. CONCLUSIONS: Extracorporeal membrane oxygenation provides an excellent environment for complex tracheal reconstruction and promotes postoperative healing by minimizing trauma to the reconstructed airway.

Video-assisted ductal ligation in premature infants.

BACKGROUND: Video-assisted thoracic surgery has been shown to be a safe and effective method of closing the patent ductus arteriosus in infants and children. We have applied this technique in low birth weight premature infants and now report our experience. METHODS: Since 1996, we have used video-assisted thoracic surgery ligation as the treatment of choice for all patent ductus arteriosus, including 100 performed on premature infants (23 to 31 weeks' gestation, mean 25.6 weeks; 0.420 to 1.5 kg, mean 0.859 kg). A modification of our previously described technique was used with a three-port approach. All patients had some degree of symptoms of congestive failure with failure to wean from ventilatory support or oxygen dependency. Five infants had associated patent foramen, and 1 had a small ventricular septal defect. RESULTS: All 100 procedures were performed in the operating room. One infant was found to have a coarctation, and the procedure was aborted. The remaining 99 were successfully ligated, although three were converted to an open procedure (3%) because of coagulopathy, poor pulmonary compliance, or hemodynamic instability. There were no procedure-related deaths; however, 15 infants subsequently died of complications of prematurity, including enterocolitis, sepsis, and late respiratory failure. Six infants had chest tubes left in place for coagulopathy, effusions, suspected air leak, and existing empyema. There were six residual pneumothoraces, four requiring treatment. CONCLUSIONS: Video-assisted thoracic surgery is a safe and effective technique for patent ductus arteriosus ligation in premature infants, including those with very low and extremely low birth weight.

Cavernous sinus thrombosis complicating sinusitis.

BACKGROUND: Septic cavernous sinus thrombosis is a rare complication of paranasal sinusitis. OBJECTIVE: To familiarize the clinician with the pathogenesis, diagnosis, and appropriate management of septic cavernous sinus thrombosis. DESIGN: Case report and literature review. SETTING: Pediatric intensive care unit in a university hospital. PATIENT: We present a 12-yr-old female with a 1 wk history of an upper respiratory tract infection with worsening dyspnea, cough, and swelling of the left eye progressing to adult respiratory distress syndrome. Secondary to the need for significant mechanical ventilatory support, venovenous extracorporeal membrane oxygenation was initiated. Computed tomography scan of the head and neck with contrast revealed bilateral cavernous sinus thrombosis. After broad-spectrum intravenous antibiotics and aggressive supportive care in conjunction with surgical intervention (maxillary sinus lavage and right orbital exploration) and anticoagulation therapy, the patient recovered. Blood cultures were positive for Viridans streptococcus. At discharge 3 wks later, the patient had improved, but had right-eye blindness. CONCLUSIONS: The diagnosis of septic cavernous sinus thrombosis requires a high index of suspicion and confirmation by imaging; early diagnosis and surgical drainage of the underlying primary source of infection in conjunction with long-term intravenous antibiotic therapy are critical for an optimal clinical outcome.

Management of venous bullet embolus in a child.

Bullet embolus is a rare complication of penetrating missile injury in children with only a handful of case reports. We describe a seven year old with a venous bullet embolus to the right ventricle.

Venovenous extracorporeal life support improves survival in adult trauma patients with acute hypoxemic respiratory failure: a multicenter retrospective cohort study.

BACKGROUND: Venovenous extracorporeal life support (VV ECLS) has been reported in adult trauma patients with severe respiratory failure; however, ECLS is not available in many trauma centers, few trauma surgeons have experience initiating ECLS and managing ECLS patients, and there is currently little evidence supporting its use in severely injured patients. This study seeks to determine if VV ECLS improves survival in such patients. METHODS: Data from two American College of Surgeons-verified Level 1 trauma centers, which maintain detailed records of patients with acute hypoxemic respiratory failure (AHRF), were evaluated retrospectively. The study population included trauma patients between 16 years and 55 years of age treated for AHRF between January 2001 and December 2009. These patients were divided into two cohorts as follows: patients who received VV ECLS after an incomplete or no response to other rescue therapies (ECLS) versus patients who were managed with mechanical ventilation (CONV). The primary outcome was survival to discharge, and secondary outcomes were intensive care unit and hospital length of stay (LOS), total ventilator days, and rate of complications requiring intervention. RESULTS: Twenty-six ECLS patients and 76 CONV patients were compared. Adjusted survival was greater in the ECLS group (adjusted odds ratio, 0.193; 95% confidence interval, 0.042-0.884; p = 0.034). Ventilator days, intensive care unit LOS, and hospital LOS did not differ between the groups. ECLS patients received more blood transfusions and had more bleeding complications, while the CONV patients had more pulmonary complications. A cohort of 17 ECLS and 17 CONV patients matched for age and lung injury severity also demonstrated a significantly greater survival in the ECLS group (adjusted odds ratio, 0.038; 95% confidence interval, 0.004-0.407; p = 0.007). CONCLUSION: VV ECLS is independently associated with survival in adult trauma patients with AHRF. ECLS should be considered in trauma patients with AHRF when conventional therapies prove ineffective; if ECLS is not readily available, transfer to an ECLS center should be pursued. LEVEL OF EVIDENCE: Therapeutic study, level III.

Neonatal cardiovascular physiology.

The pediatric surgeon deals with a large number and variety of congenital defects in neonates that frequently involve early surgical intervention and care. Because the neonatal cardiac physiology is unique, starting with the transition from fetal circulation and including differences in calcium metabolism and myocardial microscopic structure and function, it serves the pediatric surgeon well to have a sound understanding of these principles and how they directly and indirectly affect their plans and treatments. In addition, many patients will have associated congenital heart disease that can also dramatically influence not only the surgical and anesthetic care but also the timing and planning of procedures. Finally, the pediatric surgeon is often called upon to treat conditions and complications associated with complex congenital heart disease such as feeding difficulties, bowel perforations, and malrotation in heterotaxy syndromes. In this article, we will review several unique aspects of neonatal cardiac physiology along with the basic physiology of the major groups of congenital heart disease to better prepare the training and practicing pediatric surgeon for care of these complex and often fragile patients.

Neonatal coarctation repair with a long isthmus: the isthmus patch.

This case report illustrates a ductal-dependent coarctation repair in a neonate whose long isthmus was believed to make conventional end-to-end repair problematic. The isthmus and left subclavian artery were isolated and augmented with a homograft while flow to the descending aorta was preserved through the ductus. After patch augmentation of the isthmus, ductal tissue was resected and an end-to-end anastomosis was performed using the length of the augmented isthmus. Angiography 18 months later showed excellent growth of the arch despite homograft tissue comprising the majority of the isthmus at the time of repair.

Lessons learned from a single center's experience with 134 donation after cardiac death donor kidney transplants.

BACKGROUND: Reports of kidney transplantation from donation after cardiac death (DCD) donors describe high rates of delayed graft function (DGF). STUDY DESIGN: From April 1, 2003 to October 17, 2010, we performed 134 kidney transplants from DCD donors including 120 (90%) from standard-criteria donors (SCDs) and 14 (10%) from expanded-criteria donors (ECDs). Nineteen kidneys were recovered from donors managed with extracorporeal interval support for organ retrieval (EISOR) after cardiac arrest to minimize ischemic injury. RESULTS: Comparison of donor and recipient characteristics found no differences for cases managed with or without EISOR. Overall actuarial patient survival rates were 93%, 91%, and 89% at 1, 3, and 5 years, respectively, with a mean follow-up of 31 months. Overall actuarial kidney graft survival rates were 89%, 76%, and 76% at 1, 3, and 5 years, respectively. Actuarial graft survival rates of DCD ECD kidneys were 58% and 48% at 1 and 3 years, compared with 90% and 79% at 1 and 3 years for non-ECD grafts (p = 0.013). DGF occurred in 73 patients (54%) overall and was reduced from 55% to 21% (p = 0.016) with the use of EISOR in locally recovered kidneys. The mean resistance value on machine perfusion and the mean estimated glomerular filtration rate 1 month after transplantation were both improved (p < 0.05) in kidneys from donors managed with EISOR. Mean initial hospital stay was reduced from 8.0 to 5.0 days in patients receiving kidneys recovered with EISOR (p = 0.04). CONCLUSIONS: EISOR is associated with a lower rate of DGF, lower graft resistance on machine perfusion, and shorter initial hospitalization. Kidneys from DCD SCDs have excellent medium-term outcomes and represent an important means of expanding the donor pool. Kidneys from DCD ECDs have inferior outcomes.

Sinus of Valsalva aneurysm repair with partial allograft, and 10-year follow-up.

Sinus of Valsalva aneurysms are rare congenital or acquired defects that frequently are manifested by rupture into adjacent cardiac structures. Most aneurysms involve a portion of the sinus, and can be surgically treated with primary or patch closure of the defect. We present a case report of a giant aneurysm that involved the entire noncoronary sinus of Valsalva, and describe a previously unreported technique with 10-year follow-up using a partial aortic allograft to reconstruct the abnormal aortic sinus, preserving the native valve leaflets and normal sinus geometry and averting use of prosthetic materials.