A Randomized Pilot Trial Assessing the Role of Human Fibrinogen Concentrate in Decreasing Cryoprecipitate Use and Blood Loss in Infants Undergoing Cardiopulmonary Bypass.

The objective of this study was to determine whether treatment with human fibrinogen concentrate decreases the need for component blood therapy and blood loss in neonate and infant patients undergoing cardiopulmonary bypass. Pediatric patients (N = 30) undergoing elective cardiac surgery were randomized to receive human fibrinogen concentrate or placebo following cardiopulmonary bypass termination. The primary endpoint was the amount of cryoprecipitate administered. Secondary endpoints included estimated blood loss during the 24 h post-surgery; perioperative blood product transfusion; effects of fibrinogen infusion on global hemostasis, measured by laboratory testing and rotational thromboelastometry; and adverse events. No clinically significant differences were identified in baseline characteristics between groups. A significantly lower volume of cryoprecipitate was administered to the treatment group during the perioperative period [median (interquartile range) 0.0 (0.0-0.0) cc/kg vs 12.0 (8.2-14.3) cc/kg; P < 0.0001] versus placebo. No difference was observed between treatment groups in blood loss, laboratory coagulation tests, use of other blood components, or incidence of adverse events. FIBTEM amplitude of maximum clot firmness values was significantly higher among patients treated with human fibrinogen concentrate versus placebo (P ≤ 0.0001). No significant differences were observed in post-drug HEPTEM, INTEM, and EXTEM results. Human fibrinogen concentrate (70 mg/kg) administered after the termination of cardiopulmonary bypass reduced the need for transfusion with cryoprecipitate in a neonate and infant patient population.ClinicalTrials.gov identifier: NCT02822599.

A multicenter study to evaluate the pharmacokinetics and safety of liposomal bupivacaine for postsurgical analgesia in pediatric patients aged 6 to less than 17 years (PLAY).

STUDY OBJECTIVE: To evaluate the pharmacokinetics and safety of liposomal bupivacaine in pediatric patients undergoing spine or cardiac surgery. DESIGN: Multicenter, open-label, phase 3, randomized trial (PLAY; NCT03682302). SETTING: Operating room. PATIENTS: Two separate age groups were evaluated (age group 1: patients 12 to <17 years undergoing spine surgery; age group 2: patients 6 to <12 years undergoing spine or cardiac surgery). INTERVENTION: Randomized allocation of liposomal bupivacaine 4 mg/kg or bupivacaine hydrochloride (HCl) 2 mg/kg via local infiltration at the end of spine surgery (age group 1); liposomal bupivacaine 4 mg/kg via local infiltration at the end of spine or cardiac surgery (age group 2). MEASUREMENTS: The primary and secondary objectives were to evaluate the pharmacokinetics (eg, maximum plasma bupivacaine concentrations [Cmax], time to Cmax) and safety of liposomal bupivacaine, respectively. MAIN RESULTS: Baseline characteristics were comparable across groups. Mean Cmax after liposomal bupivacaine administration was lower versus bupivacaine HCl in age group 1 (357 vs 564 ng/mL); mean Cmax in age group 2 was 320 and 447 ng/mL for spine and cardiac surgery, respectively. Median time to Cmax of liposomal bupivacaine occurred later with cardiac surgery versus spine surgery (22.7 vs 7.4 h). In age group 1, the incidence of adverse events (AEs) was comparable between liposomal bupivacaine (61% [19/31]) and bupivacaine HCl (73% [22/30]). In age group 2, 100% (5/5) and 31% (9/29) of patients undergoing spine and cardiac surgery experienced AEs, respectively. AEs were generally mild or moderate, with no discontinuations due to AEs or deaths. CONCLUSIONS: Plasma bupivacaine levels following local infiltration with liposomal bupivacaine remained below the toxic threshold in adults (~2000-4000 ng/mL) across age groups and procedures. AEs were mild to moderate, supporting the safety of liposomal bupivacaine in pediatric patients undergoing spine or cardiac surgery. Clinical trial number and registry URL: ClinicalTrials.gov identifier: NCT03682302.

Effectiveness of Liposomal Bupivacaine Compared With Standard-of-Care Measures in Pediatric Cardiothoracic Surgery: A Retrospective Cohort Study.

OBJECTIVE: Effective postsurgical pain management is important for pediatric patients to improve outcomes while reducing resource use and waste. The authors examined opioid consumption and economic outcomes associated with liposomal bupivacaine (LB) or non-LB analgesia use in pediatric patients undergoing cardiothoracic surgery. DESIGN: The authors retrospectively analyzed Premier Healthcare Database records. SETTING: The data extracted from the database included patient records from hospitals across the United States in both rural and urban locations. PARTICIPANTS: The records included data from patients aged 12-to-<18 years. INTERVENTIONS: The records belonged to patients undergoing video-assisted thoracoscopic procedures (VATS) who received LB or non-LB analgesia after surgery. MEASUREMENTS AND MAIN RESULTS: Outcomes included in-hospital postsurgical opioid consumption in morphine milligram equivalents (MMEs), hospital length of stay (LOS), and total hospital costs; the LB and non-LB cohorts were compared using a generalized linear model with inverse probability of treatment weighting to balance the cohorts. For VATS procedures, pediatric patients receiving LB had significant reductions in in-hospital opioid consumption (632 v 991 MMEs; p < 0.0001), shorter LOS (5.1 v 5.6 days; p = 0.0023), and lower total hospital costs ($18,084 v $21,962; p < 0.0001) compared with those receiving non-LB analgesia. CONCLUSIONS: These results support use of LB in multimodal analgesia regimens for managing pain in pediatric patients after cardiothoracic surgery.

Liposomal Bupivacaine Infiltration After Median Sternotomy in Pediatric Cardiac Surgery.

OBJECTIVE: The present study retrospectively ascertained whether liposomal bupivacaine (LB) injected subcutaneously after median sternotomy incisions in pediatric cardiac surgery patients is as efficacious as the ON-Q PainBuster pump (ON-Q) (Avonas Medical, Alpharetta, GA). DESIGN: Retrospective cohort comparison. SETTING: Pediatric hospital. PARTICIPANTS: Cardiac surgery patients who were treated with LB for elective cardiac surgery. INTERVENTIONS: Patients received 4 mg/kg of LB admixed with 0.25% bupivacaine and 0.9% normal saline. These patients were compared with an age- and procedure-matched control group of similar size treated with the ON-Q pump (continuous infusion 0.25% bupivacaine via subcutaneous catheter). Total analgesics used and route, other analgesics or sedatives, and pain scores (first 24 hours and cumulative) were tracked for 96 hours after surgery. MEASUREMENTS AND MAIN RESULTS: A total of 222 patients were equally divided between the two groups. Overall, the median (interquartile range) age was 6.5 (3.8-12.7) years. Unadjusted analysis suggested that patients in the LB group were administered a significantly higher dose of intravenous acetaminophen (77.4 v 60.0 mg/kg; p < 0.05). Extubation in the operating room was significantly higher in the LB patients (p < 0.05). Narcotic (morphine) administration was significantly higher in the ON-Q group (100.0% v 95.5%; p < 0.05). Although the median pain score within the first 24 hours was higher in LB patients (27.0 v 17.0; p < 0.05), there was a significantly greater difference observed in the Numeric Rating Scale area under the curve for the ON-Q group. CONCLUSIONS: LB is at least as effective as the ON-Q is for providing analgesia after median sternotomy incision in children.

Three-year experience with immediate extubation in pediatric patients after congenital cardiac surgery.

BACKGROUND: In pediatric cardiac anesthesiology, there is increased focus on minimizing morbidity, ensuring optimal functional status, and using health care resources sparingly. One aspect of care that has potential to affect all of the above is postoperative mechanical ventilation. Historically, postoperative ventilation was considered a must for maintaining patient stability. Ironically, it is recognized that mechanical ventilation may increase risk of adverse outcomes in the postoperative period. Hence, many institutions have advocated for immediate extubation or early extubation after many congenital heart surgeries which was first reported decades ago. METHODS: 637 consecutive patient charts were reviewed for pediatric patients undergoing cardiac surgery with cardiopulmonary bypass. Patients were placed into three groups. Those that were extubated in the operating room (OR) at the conclusion of surgery (Immediate Extubation or IE), those that were extubated within six hours of admission to the ICU (Early Extubation or EE) and those that were extubated sometime after six hours (Delayed Extubation or DE). Multiple variables were then recorded to see which factors correlated with successful Immediate or Early Extubation. RESULTS: Overall, 338 patients (53.1%) had IE), 273 (42.8%) had DE while only 26 patients (4.1%) had EE. The median age was 1174 days for the IE patients, 39 days for the DE patients, whereas 194 days for EE patients (p < 0.001). Weight and length were also significantly different in at least one extubation group from the other two (p < 0.001). The median ICU LOS was 3 and 4 days for IE and EE patients respectively, whereas it was 9.5 days for DE patients (p < 0.001). DE group had a significant longer median anesthesia time and cardiopulmonary bypass time than the other two extubation groups (p > 63,826.88 < 0.001). Regional low flow perfusion, deep hypothermia, deep hypothermic circulatory arrest, redo sternotomy, use of other sedatives, furosemide, epinephrine, vasopressin, open chest, cardiopulmonary support, pulmonary edema, syndrome, as well as difficult intubation were significantly associated with delayed extubation (IE, EE or DE). CONCLUSIONS: Immediate and early extubation was significantly associated with several factors, including patient age and size, duration of CPB, use of certain anesthetic drugs, and the amount of blood loss and blood replacement. IE can be successfully accomplished in a majority of pediatric patients undergoing surgery for congenital heart disease, including in a minority of infants.

Interval changes in ROTEM values during cardiopulmonary bypass in pediatric cardiac surgery patients.

INTRODUCTION: Rotational thromboelastometry (ROTEM) has been shown to reduce the need for transfused blood products in adult and pediatric cardiac surgery patients. However, similar evidence in newborns, neonates, and young infants is lacking. We quantified ROTEM value changes in pediatric patients on cardiopulmonary bypass (CPB) before, during and after blood product transfusion. METHODS: Each surgery had at least four interventions: initiating CPB; platelet administration during rewarming phase; post-CPB and following protamine and human fibrinogen concentrate (HFC) administration; and further component therapy if bleeding persisted and ROTEM indicated a deficiency. ROTEM assays were performed prior to surgery commencement, on CPB prior to platelet administration and following 38 mL/kg platelets, and post-CPB after protamine and HFC administration. ROTEM assays were also performed in the post-CPB period after further blood component therapy administration. RESULTS: Data from 161 patients were analyzed. Regression models suggested significant changes in HEPTEM clotting time after all interventions. PLT administration during CPB improved HEPTEM α by 22.1° (p < 0.001) and FIBTEM maximum clot firmness (MCF) by 2.9 mm (p < 0.001). HFC administration after CPB termination significantly improved FIBTEM MCF by 2.6 mm (p < 0.001). HEPTEM MCF significantly increased after 3/4 interventions. HEPTEM α significantly decreased after two interventions and significantly increased after two interventions. Greatest perturbances in coagulation parameters occurred in patients ≤90 days of age. CONCLUSION: CPB induced profound perturbations in ROTEM values in pediatric cardiac surgery patients. ROTEM values improved following PLT and HFC administration. This study provides important clinical insights into ROTEM changes in pediatric patients after distinct interventions.

Correlation Between ROTEM FIBTEM Maximum Clot Firmness and Fibrinogen Levels in Pediatric Cardiac Surgery Patients.

This study evaluated whether rotational thromboelastometry (ROTEM; Tem International GmbH, Munich, Germany) FIBTEM maximum clot firmness (MCF) can be used to predict plasma fibrinogen level in pediatric patients undergoing cardiac surgery. Linear regression was conducted to predict plasma fibrinogen level using FIBTEM MCF (0.05 level of significance). Scatter plot with the regression line for the model fit was created. Fifty charts were retrospectively reviewed, and 87 independent measurements of FIBTEM MCF paired with plasma fibrinogen levels were identified for analysis. Linear regression analysis suggested a significant positive linear relationship ( P < .0001) between plasma fibrinogen levels and MCF. Both MCF intercept and slope were significantly correlated with fibrinogen level ( P < .0001). The estimated regression equation (predicted fibrinogen = 78.6 + 12.4 × MCF) indicates that a 1-mm increase in MCF raises plasma fibrinogen level by an average of 12.4 mg/dL. The statistically significant positive linear relationship observed between MCF and fibrinogen levels ( P < .001) suggests that MCF can be used as a surrogate for fibrinogen level. This relationship is of clinical relevance in the calculation of patient-specific dosing of fibrinogen supplementation in this setting.

Non-invasive cardiac output monitor validation study in pediatric cardiac surgery patients.

STUDY OBJECTIVE: To validate a non-invasive cardiac output monitor in pediatric cardiac surgery patients. DESIGN: Prospective trial. SETTING: Operating room. PATIENTS: 20 pediatric cardiac surgery patients ASA physical status 3 and 4. INTERVENTIONS: Aesculon noninvasive cardiac output monitor was used and compared to the cardiac output derived from the Fick equation. Oxygen consumption was measured and blood samples were taken from the arterial line and from the superior and inferior vena cava. MEASUREMENTS: Noninvasive cardiac output and cardiac index and Fick cardiac output and cardiac index. Oxygen consumption was measured by the TreyMed metabolic monitor. Blood samples were simultaneously drawn from the arterial line and from the superior and inferior vena cava purse string sites by the surgeon, prior to commencing cardiopulmonary bypass. Another data set was obtained right after termination of cardiopulmonary bypass. RESULTS: There was a direct, significant relationship between Fick CO/CI and NICOM CO/CI measurements. More dispersion is detected when the magnitude of the measure increases, i.e., for older and larger patients. CONCLUSIONS: There is a strong correlation between the cardiac output values derived from the Fick equation and the Aesculon non-invasive cardiac output monitor.

Dexmedetomidine use in patients undergoing electrophysiological study for supraventricular tachyarrhythmias.

BACKGROUND: Dexmedetomidine is a selective alpha-2 adrenergic agonist with sedative, analgesic, and anxiolytic properties. Dexmedetomidine has not been approved for use in pediatrics. Dexmedetomidine has been reported to depress sinus node and atrioventricular nodal function in pediatric patients; it has been suggested that the use of dexmedetomidine may not be desirable during electrophysiological studies. AIM: We hypothesize that the use of dexmedetomidine does not inhibit the induction of supraventricular tachyarrhythmias (SVT) during electrophysiological studies and does not inhibit the ablation of such arrhythmias. METHODS: In this retrospective, observational cohort study, we reviewed all cases presenting to the cardiac catheterization laboratory for diagnosis or treatment of SVT since 2007. All cases were performed by the same electrophysiologist. The anesthesia was provided by one of the three cardiac anesthesiologists. One cardiac anesthesiologist did not use dexmedetomidine during electrophysiological studies. A second used dexmedetomidine, but only with an infusion. The third used dexmedetomidine with a primary bolus and an infusion. Thus, the patients were stratified into three different groups: Group 1 patients did not receive any dexmedetomidine. Group 2 patients received a dexmedetomidine infusion of 0.5-1 µg·kg-1 ·h-1 . Group 3 patients received a dexmedetomidine infusion of 0.5-1 µg·kg-1 ·h-1 and a dexmedetomidine bolus prior to the infusion of 0.5-1 µg·kg-1 . We then compared those patients for the following variables: demographic data including age, sex, height, weight; anesthetic data such as, mask vs intravenous induction, identity of induction agent, amount of sevoflurane and propofol used; amount of dexmedetomidine used; presence of congenital heart disease and other comorbidities; the need for isoproterenol and dose, the need for adenosine and dose, and the need for any other medications to affect rhythm both before and after radiofrequency ablation; the ability to induce the arrhythmia, the type of arrhythmia, the presence of Wolff-Parkinson-White syndrome, the presence of an accessory pathway, the ablation rate, and the recurrence rate. RESULTS: There was no difference in the anesthetic agents, except there was a lesser amount of propofol used in the dexmedetomidine groups (χ2(2) = 48.2, P < 0.001). There was no difference in the electrophysiological parameters among groups, except the Group 1 patients did require the use of isoproterenol in the preablation period less often compared to the dexmedetomidine groups (χ2(2) = 15.2, P < 0.01). However, with the greater use of isoproterenol, there was no difference in the ability to induce the arrhythmia. Moreover, the percentage of patients ablated, and the recurrence rate among groups was the same. CONCLUSIONS: We conclude that dexmedetomidine does not interfere with the conduct of electrophysiological studies for SVT and the successful ablation of such arrhythmias. However, dexmedetomidine use did result in a greater need for isoproterenol.

Comparison of point-of-care activated clotting time systems utilized in a single pediatric institution.

This study compares four different activated clotting time (ACT) point-of-care (POC) testing systems used at our institution for the management of patients undergoing heparin therapy. We evaluated these systems under identical conditions to determine their accuracy, reproducibility, ease of use, and cost. Two separate testing stations containing four ACT systems were used. The testing order was randomized for every sample and performed by two trained individuals. Samples of fresh heparinized whole blood were taken at regular intervals and distributed to each station. Each operator tested 50 samples, totaling 400 ACT tests. The ACT value was significantly affected by the type of machine used at both stations 1 and 2 (p < .001). Compared with all systems, the Medtronic ACT Plus Automated Coagulation Timer System (ACT Plus) resulted in the most consistent ACT values (median = 171, Interquartile Range (IQR): 169-175) and least variability (172.17 +/- 5.24). The Hemochron Signature Elite Whole Blood Microcoagulation System had the most variability (221.10 +/- 14.78) and yielded consistently higher ACT values (median = 220, IQR: 210-229.5) compared with other systems. The ACT values reported by the i-STAT Handheld and Test Cartridge Blood Analysis System (153.30 +/- 7.87) were consistently lower (median = 154, IQR: 147-161) in comparison to the ACT Plus and Medtronic HMS Plus Hemostasis Management System (180.60 +/- 7.60, median = 181, IQR: 175-186). There was no statistical difference in results between the two testing sites (p > .05) or the operators (p > .05). The significant finding of this study was the affect each system has on the ACT value. This investigation demonstrates the variability that exists among different ACT monitoring systems at our institution. The discrepant variation in ACT values that exists with the Hemochron system questions the reliability of its use in the management of patients undergoing heparin therapy.

Continuous incisional infusion of local anesthetic in pediatric patients following open heart surgery.

AIM: To determine the efficacy and safety of a continuous subcutaneous local anesthetic (LA) infusion in pediatric patients following open heart surgery. BACKGROUND: The use of a continuous LA infusion has been shown to be beneficial following adult cardiac surgery. To date there are no studies in the pediatric population. METHODS/MATERIALS: Using a prospective, randomized, and double blind design, we compared LA, either 0.25% levobupivacaine or bupivacaine (Treatment Group) to saline (Placebo Group) delivered subcutaneously via a continuous infusion for 72 h after open heart surgery in 72 patients. Requirements for postoperative analgesics and pain scores were recorded for 72 h and plasma levels of local anesthetic were measured. Secondary outcomes measures included time to first oral intake, time to first bowel movement, time to urinary catheter removal, length of stay, requirements for antiemetics and additional sedation. RESULTS: Total morphine requirements over the first 24 h were less in the Treatment Group than the Placebo Group (0.05 mg x kg(-1) vs 0.2 mg x kg(-1), P = 0.007); this was true for all patient groups except those patients weighing less than 6.3 kg. The number of patients requiring no morphine was greater in the Treatment Group (7/35 vs 1/37, P = 0.02). The Treatment Group also received less midazolam, lorazepam, and ketorolac than the Placebo Group over 72 h due to the reduced clinical need for these agents in patients weighing less than 31 kg. There were no differences in secondary outcomes. CONCLUSIONS: A continuous incisional infusion of LA reduced postoperative analgesic requirement and sedative use in pediatric patients undergoing a median sternotomy incision. Dosed at a maximum rate of 0.4 mg x kg(-1) x h(-1), a continuous incisional infusion of LA is effective and safe for up to 72 h, with plasma levels of local anesthetic well below the toxic threshold.

Hybrid ventricular decompression in pulmonary atresia with intact septum.

Initial palliation for pulmonary atresia with intact ventricular septum continues to evolve in the face of significant early and late morbidity. In patients with suitable anatomy, decompression of the right ventricle may be the first step in treatment. A hybrid approach to right ventricular decompression, combining surgery and interventional catheterization techniques is described. Direct access to the right ventricle through a subxiphoid incision with transventricular sheath placement is used to provide optimum catheter position for radiofrequency perforation of membranous pulmonary atresia followed by balloon dilation. The technique may address key limitations of the traditional surgical and interventional approaches.

Anaesthetic complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease.

Congenital heart defects are the most common cause of death in infants and young children in the developed world. As the mortality in this population has declined to less than 5%, more attention is being focused now on reducing post-procedural morbidities that may seriously impact the patient and their families. Because of multiple reasons, paediatric cardiac surgery and anaesthesia is a perfect model for studying human errors and their impact on patient safety. Congenital cardiac disease is a common lesion causing much morbidity, pain, and loss of life. Over 44,000 surgical procedures are performed yearly to repair congenital cardiac problems in the United States alone. The reduction or elimination of iatrogenic adverse outcomes, given the current mortality rates of 4.2%-4.5%, might lead to as many as 500 children achieving better outcomes or shorter hospitalizations.Efforts to quantify the frequency of complications related to anaesthesia in patients undergoing congenital cardiac surgery have been difficult to date because of the low occurrence of this surgery compared to other surgeries on children and the relatively rare incidence of complications related to anaesthesia in this population. Anaesthesiologists play a crucial role in the reduction, recognition, and timely treatment of medical errors that impact this morbidity. Paediatric cardiac surgery encompasses many complex procedures that are highly dependent upon a sophisticated organizational structure, effective communication, coordinated efforts of multiple individuals working as a team, and high levels of cognitive and technical performance. Human factor error analysis in this patient population has shown how frequently both minor and major errors occur. The goal of this paper is to outline the frequency and sources of these errors and to suggest treatment strategies which may minimize their occurrence.

Initial experience with dexmedetomidine for diagnostic and interventional cardiac catheterization in children.

BACKGROUND: Children undergoing diagnostic and interventional cardiac catheterization require deep sedation or general anesthesia (GA). Dexmedetomidine, a selective alpha-2 adrenergic agonist, has sedative, analgesic and anxiolytic properties without respiratory depression. These characteristics make it potentially suitable as a sedative agent during diagnostic procedures in children. We report our experience using dexmedetomidine in 20 children aged 3 months to 10 years undergoing cardiac catheterization. METHODS: Following a midazolam premedication, intravenous access was secured facilitated by the inhalation of sevoflurane in oxygen. A loading dose of 1 microg x kg(-1) dexmedetomidine was administered over 10 min followed by an initial infusion rate of 1 microg x kg(-1) x h(-1). Nasal cannulae were applied, allowing endtidal CO2 monitoring with the patients breathing spontaneously. Hemodynamic parameters, Bispectral Index Score (BIS) and sedation score were measured every 5 min. Patient movement or evidence of inadequate sedation were treated with propofol (1 mg x kg(-1)). The dexmedetomidine infusion rate was titrated to the level of sedation to a maximum of 2 microg x kg(-1) x h(-1) to maintain a sedation score of 4-5 and a BIS value <80. RESULTS: Five patients (25%) had some movement on local infiltration or groin vessel access. This did not necessitate restraint or result in difficulty securing vascular access. No patients failed sedation that required the addition of another sedative agent or conversion to GA; eight patients were sedated with dexmedetomidine alone; however, 12 (60%) patients did receive a propofol bolus at some time during the procedure due to movement, increasing BIS value or in anticipation of stimulation. There were no incidences of airway obstruction or respiratory depression. In all cases the heart rate and blood pressure remained within 20% of baseline. No patient required treatment for profound bradycardia or hypotension. The average infusion rate for dexmedetomidine following the loading dose was 1.15 (+/-0.29)microg x kg(-1) x h(-1) (range 0.6-2.0 microg x kg(-1) x h(-1)). CONCLUSIONS: This initial experience showed dexmedetomidine, with or without the addition of propofol, may be a suitable alternative for sedation in spontaneously breathing patients undergoing cardiac catheterization.

Diminished Blalock-Taussig shunt flow detected by cerebral oximetry.

Near-infrared spectroscopy is a noninvasive optical technique used to monitor brain tissue oxygenation. Measurement of cerebral oxygenation is a more sensitive monitor of oxygen delivery to the brain than other available monitors. Prolonged cerebral desaturation is correlated with poor neurological outcomes. We report a case where the cerebral oximeter alerted us to diminished blood flow in a preexisting Blalock-Taussig (subclavian artery to pulmonary artery) shunt.

Anesthetic management of conjoined twins presenting for palliative open-heart surgery.

A set of dicephalous parapagus twins was born at 36 wk gestational age. Twin A had complex congenital heart disease in the form of a single ventricle that would be fatal without surgical intervention. Twin B had normal intracardiac anatomy. The twins were deemed surgically inseparable and the parents requested palliative open-heart surgery on Twin A. The twins shared a common circulatory system to the lower half of the body so that physiological manipulations in one twin adversely affected the other's hemodynamic condition. Twin A underwent successful single ventricle palliation; however, after a prolonged hospitalization, the infants died secondary to severe respiratory disease in Twin B.