Faculty Bibliography

Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.

OBJECTIVES:The transthoracic intracardiac line placed in the right atrium provides a convenient access to the central venous system following cardiac surgery. However, it is associated with risks such as migration and bleeding. We conducted a retrospective study to determine whether position of transthoracic line with respect to site of exit from the chest makes a difference in the rate of complications. METHODS:All infants receiving a transthoracic intracardiac line in the right atrium following cardiac surgery between June 2012 and December 2015 were part of the study. A 3.5-Fr double-lumen umbilical venous catheter was placed directly into the right atrium. The lines exited the thorax either above in the suprasternal notch (upper transthoracic line) or below the diaphragm across the abdominal wall (lower transthoracic line). Patients were analysed for complications such as catheter migration, bleeding upon removal, atrial thrombus, line occlusion, premature removal and failed removal. RESULTS:A total of 131 patients received a transthoracic intracardiac line during the study period. Of the total patients, 88 patients received the upper transthoracic line and 43 patients received the lower transthoracic line. The upper transthoracic line was associated with significantly lower incidence of catheter migration (1% vs 14%) and this held by multivariable logistic regression, adjusting for age and duration of the line (P = 0.003). There was no difference in the rate of other complications including bleeding. CONCLUSIONS:The upper transthoracic line is associated with significantly lower incidence of catheter migration and offers a more optimum position for central access following cardiac surgery.

OBJECTIVE:Medically refractory chylous pleural effusion after congenital heart surgery is associated with significant morbidity and mortality, especially in infants. We reviewed our experience with diaphragmatic fenestration procedure in this group of patients. METHODS:A retrospective chart review of all patients who had diaphragmatic fenestrations for chylous effusion at our institution over a 2-year period was performed. RESULTS:A total of 9 diaphragmatic fenestration procedures were performed in 8 patients who had failed medical management of chylous pleural effusions. All procedures except 1 were performed on the right side. The median age at time of procedure was 4.6 months (range, 3 weeks to 14 months). The average time between primary congenital cardiac surgery and fenestration was 26 days (range, 4-53 days). Three patients had single ventricle repair. Average time of chest tube removal after procedure was 4 days. Average time to extubation was 3 days. All patients but 1 were able to advance to full feedings without reaccumulation of chylous effusion within 12 days. No complications developed in the patients. Recurrent effusion over a median follow-up period of 19 months did not develop in the patients. CONCLUSIONS:Diaphragmatic fenestration is an effective and safe strategy for management of persistent chylous effusions after congenital cardiac surgery.

BACKGROUND:Numerous advances in surgical techniques and understanding of single-ventricle physiology have resulted in improved survival. We sought to determine the influence of various demographic, perioperative, and patient-specific factors on the survival of single-ventricle patients following stage 1 palliation at our institution. METHODS:We conducted a retrospective study of all single-ventricle patients who had undergone staged palliation at our institution over an 8-year period. Data were collected from the Society of Thoracic Surgeons Congenital Heart Surgery database and from patient charts. Information on age, weight at stage 1 palliation, prematurity, genetic abnormalities, non-cardiac anomalies, ventricular dominance, and type of palliation was collected. Information on mortality and unplanned reinterventions was also collected. RESULTS:A total of 72 patients underwent stage 1 palliation over an 8-year period. There were 12 deaths before and one death after stage 2 palliation. There was no hospital mortality following Glenn or Fontan procedures. On univariate analysis, low weight at the time of stage 1 palliation and prematurity were found to be risk factors for mortality following stage 1 palliation. However, multivariable Cox regression analysis revealed weight at stage 1 palliation to be a strong predictor of mortality. The type of stage 1 palliation did not have any influence on the outcome. No difference in survival was noted following the Glenn procedure. CONCLUSION/CONCLUSIONS:Low weight has a deleterious impact on survival following stage 1 palliation. This is mitigated by stage 2 palliation. The type of stage 1 palliation itself has no bearing on the outcome.

Studies in adult HT have demonstrated improved cardiac function in the recipient following administration of T3 to the donor. The purpose of this experiment was to assess the effects of T3 on the function of the immature donor heart following HT in a piglet model. A total of 32 piglets were divided into 16 donors and 16 recipients. Following creation of brain death, half of the donor piglets were randomized to receive three doses of T3 (0.2 μg/kg) along with hydrocortisone (1 mg/kg). The donor hearts were then transplanted into the recipient piglets on CPB. Duration of survival off CPB, inotrope score, and EF of heart following CPB were evaluated. There were no differences between the two groups in age, weight, pre-brain death EF, T3 levels, and CPB times. Post-CPB survival times were inversely related to the ischemic times in both groups (Pearson r=-0.80, P<.001), and this relationship was not influenced by T3. There was no difference in inotrope score, EF, or biochemical assessment between the two groups. Administration of T3 in combination with hydrocortisone to the brain-dead donor confers no beneficial effect on myocardial function or survival following HT in a piglet model.

Extracorporeal membrane oxygenation was instituted as an aid to in-hospital cardiopulmonary resuscitation (E-CPR) nearly 23 years ago, this led to remarkable improvement in survival considering the mortality rate associated with conventional cardiopulmonary resuscitation (CPR). Given this success, one begins to wonder whether the time has come for expanding the use of E-CPR to outside hospital cardiac arrests especially in the light of development of newer extracorporeal life support devices that are small, mobile, and easy to assemble. This editorial will review recent studies on this subject and address some key guidelines and limitations of this evolving and promising technology.

A 9-year-old child presented with syncope during exercise. He received a diagnosis of congenital atresia of the left main coronary artery by angiography. He underwent successful coronary artery bypass grafting. On the third postoperative day, he experienced acute, precordial chest pain. An urgent computed tomographic scan showed an unrecognized anomalous origin of the right coronary artery (RCA) with a 1.5-cm intramural course. He was taken back to the operating room to undergo unroofing of the RCA. This case highlights the difficulty involved in making two rare diagnoses that can cause the same exact symptoms in a patient and the surgical challenges associated with it.

BACKGROUND:The polytetrafluoroethylene tube used as right ventricle to pulmonary artery conduit in the stage 1 Norwood operation is associated with risks of suboptimal branch pulmonary artery growth, thrombosis, free insufficiency, and long-term right ventricular dysfunction. Our experience with use of valved femoral vein homograft as right ventricle to pulmonary artery conduit is described. METHODS:Between June 2012 and December 2015, 15 neonates with hypoplastic left heart syndrome or complex single ventricle underwent stage 1 Norwood operation with valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit. The median age at surgery was 3 days and the mean weight was 3 kg. The size of the femoral vein homograft was 5 mm in 8 patients and 6 mm in 7 patients. RESULTS:). No patient had thrombosis of conduit. Most femoral vein conduits remained competent in the first month after stage 1 Norwood operation, although most became incompetent by 3 months. Catheter intervention on the conduit was necessary in 7 patients. Right ventricular function was preserved in most patients at follow-up. CONCLUSIONS:The use of femoral vein homograft as right ventricle to pulmonary artery conduit in the Norwood operation is safe and associated with good pulmonary artery growth and preserved ventricular function. Balloon dilation of the conduit may be necessary during the interstage period.