Haemodynamic effects of prophylactic post-operative hydrocortisone following cardiopulmonary bypass in neonates undergoing cardiac surgery.

Multiple studies have endeavoured to define the role of steroids in paediatric congenital heart surgery; however, steroid utilisation remains haphazard. In September, 2017, our institution implemented a protocol requiring that all neonates undergoing cardiac surgery with the use of cardiopulmonary bypass receive a five-day post-operative hydrocortisone taper. This single-centre retrospective study was designed to test the hypothesis that routine post-operative hydrocortisone administration reduces the incidence of capillary leak syndrome, leads to favourable postoperative fluid balance, and less inotropic support in the early post-operative period. Data were gathered on all term neonates who underwent cardiac surgery with the use of bypass between September, 2015 and 2019. Subjects who were unable to separate from bypass, required long-term dialysis, or long-term mechanical ventilation were excluded. Seventy-five patients met eligibility criteria (non-hydrocortisone group = 52; hydrocortisone group = 23). For post-operative days 0-4, we did not observe a significant difference in net fluid balance or vasoactive inotropic score between study groups. Similarly, we saw no major difference in secondary clinical outcomes (post-operative duration of mechanical ventilation, ICU/hospital length of stay, and time from surgery to initiation of enteral feeds). In contrast to prior analyses, our study was unable to demonstrate a significant difference in net fluid balance or vasoactive inotropic score with the administration of a tapered post-operative hydrocortisone regimen. Similarly, we saw no effect on secondary clinical outcomes. Further long-term randomised control studies are necessary to validate the potential clinical benefit of utilising steroids in paediatric cardiac surgery, especially in the more fragile neonatal population.

Two rare cases of paraganglioma in patients with Fontan physiology.

Pheochromocytoma/paraganglioma is an exceedingly rare tumour, thought to share an association with cyanotic CHD. This association is thought to be a result of chronic hypoxaemia (Antonio et al, Revista Española de Cardiología (English Edition) 2017; 70: 673-675; Folger et al, Circulation 1964; 29: 750-757; Opotowsky et al, J Clin Endocrinol Metab 2015; 100: 1325-1334) We report two cases of paraganglioma over a 4-year period in patients with hypoplastic left heart syndrome who had undergone Fontan completion by ages 2 and 4. Based on a very small number of reported cases of CHD, the mechanism of tumourigenesis is unclear. It is imperative that cases associated with CHD continue to be reported so that we may learn more about the pathogenesis and epidemiology of this entity.

Alternative strategies in newborns and infants with major co-morbidities to improve congenital heart surgery outcomes at an emerging programme.

INTRODUCTION: Debilitating patient-related non-cardiac co-morbidity cumulatively increases risk for congenital heart surgery. At our emerging programme, flexible surgical strategies were used in high-risk neonates and infants generally considered in-operable, in an attempt to make them surgical candidates and achieve excellent outcomes. MATERIALS AND METHODS: Between April, 2010 and November, 2013, all referred neonates (142) and infants (300) (average scores: RACHS 2.8 and STAT 3.0) underwent 442 primary cardiac operations: patients with bi-ventricular lesions underwent standard (n=294) or alternative (n=19) repair/staging strategies, such as pulmonary artery banding(s), ductal stenting, right outflow patching, etc. Patients with uni-ventricular hearts followed standard (n=96) or alternative hybrid (n=34) staging. The impact of major pre-operative risk factors (37%), standard or alternative surgical strategy, prematurity (50%), gestational age, low birth weight, genetic syndromes (23%), and major non-cardiac co-morbidity requiring same admission surgery (27%) was analysed on the need for extracorporeal membrane oxygenation, mortality, length of intubation, as well as ICU and hospital length of stays. RESULTS: The need for extracorporeal membrane oxygenation (8%) and hospital survival (94%) varied significantly between surgical strategy groups (p=0.0083 and 0.028, respectively). In high-risk patients, alternative bi- and uni-ventricular strategies minimised mortality, but were associated with prolonged intubation and ICU stay. Major pre-operative risk factors and lower weight at surgery significantly correlated with prolonged intubation, hospital length of stay, and mortality. DISCUSSION: In our emerging programme, flexible surgical strategies were offered to 53/442 high-risk neonates and infants with complex CHDs and significant non-cardiac co-morbidity, in order to buffer risk and achieve patient survival, although at the cost of increased resource utilisation.

Unusual cause of aborted sudden cardiac death in a teen athlete: homozygosity for the 4G allele of the plasminogen activase inhibitor type 1 gene.

Common aetiologies of sudden cardiac death in children include coronary anomalies, channelopathies, and cardiomyopathies. Less frequently, hypercoagulable states cause sudden arrest. We report an unusual case of aborted sudden cardiac death in a teenager, ultimately found to have homozygosity for the 4G allele of the plasminogen activase inhibitor type 1 gene.

Chondrosarcoma presenting with pulmonary embolism in a 9-year-old girl: a case report.

Chondrosarcoma is a malignant bone tumour common in adults, third to myeloma and osteosarcoma, but is exceptionally rare in children. Here we discuss a 9-year-old girl presenting with occlusive right pulmonary artery neoplastic embolus, resulting from a primary right proximal humerus chondrosarcoma. To the best of our knowledge, this the first pediatric and only second overall case reported in the United States of a neoplastic pulmonary embolus resulting from a primary chondrosarcoma.

Angiotensin-converting enzyme inhibitor nephrotoxicity in neonates with cardiac disease.

Angiotensin-converting enzyme inhibitors (ACEi) are commonly used for pediatric cardiology patients. However, studies examining their safety for neonates with cardiac disease are scarce. The current study aimed to test the hypothesis that ACEi-mediated nephrotoxicity occurs in neonates and may be underappreciated in this population. A retrospective review of 243 neonates with cardiac disease between 2007 and 2010 was performed. Demographic data, weight, length, captopril and enalapril dosing, serum [K⁺], serum creatinine, and concomitant medications during ACEi therapy were recorded and analyzed. Body surface area (BSA), creatinine clearance (CrCl), and change in [K⁺] were calculated. The age range of neonates at ACEi initiation was 15.9-18.1 days. The inclusion criteria was met by 206 neonates: 168 term (82%) and 38 preterm (18%) newborns. Of these neonates, 42% were female, and all the patients had a BSA smaller than 0.33 m² (a group known to have relative renal insufficiency). The mean dose of enalapril was 0.08 ± 0.007 mg/kg for the preterm neonates and 0.08 ± 0.003 mg/kg for the term neonates. The mean dose of captopril was 0.07 ± 0.009 mg/kg for the preterm neonates and 0.13 ± 0.019 mg/kg for the term neonates. A significant decrease in CrCl occurred for both the preterm (p < 0.01) and term (p < 0.001) neonates while they were receiving ACEi. However, the two groups did not differ significantly (p = 0.183). Nearly 42% of all the patients showed renal risk, with approximately 30% demonstrating renal failure by modified pRIFLE (pediatric risk, injury, failure, loss, and end-stage renal disease) criteria. Despite the lack of significantly different CrCl, the premature neonates were more likely to experience ACEi-related renal failure by pRIFLE (55%) than their term counterparts (23%; p < 0.001). Despite its common use for term neonates with cardiac disease, ACEi should be used cautiously and only when indications are clear. These results also raise the question whether ACEi should be used at all for preterm neonates.

Off-pump snare technique for congenital left atrial appendage aneurysm.

Left atrial appendage aneurysm is an extremely rare anomaly and as such has been rarely imaged or seen intraoperatively with very little accumulated management experience. The available scant published literature stresses resection on cardiopulmonary bypass as the safest and by far the most commonly applied technique. We suggest a novel alternative imaging-guided management utilising an off-pump tourniquet snare technique under live transoesophageal echocardiography.

Genetic variants and effect modifiers of QT interval prolongation in patients with sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) is a common inherited blood disorder among African Americans (AA), with premature mortality which has been associated with prolongation of the heart rate-corrected QT interval (QTc), a known risk factor for sudden cardiac death. Although numerous genetic variants have been identified as contributors to QT interval prolongation in the general population, their impact on SCD patients remains unclear. This study used an unweighted polygenic risk score (PRS) to validate the previously identified associations between SNPs and QTc interval in SCD patients, and to explore possible interactions with other factors that prolong QTc interval in AA individuals with SCD. METHODS: In SCD patients, candidate genetic variants associated with the QTc interval were genotyped. To identify any risk SNPs that may be correlated with QTc interval prolongation, linear regression was employed, and an unweighted PRS was subsequently constructed. The effect of PRS on the QTc interval was evaluated using linear regression, while stratification analysis was used to assess the influence of serum alanine transaminase (ALT), a biomarker for liver disease, on the PRS effect. We also evaluated the PRS with the two subcomponents of QTc, the QRS and JTc intervals. RESULTS: Out of 26 candidate SNPs, five risk SNPs were identified for QTc duration under the recessive model. For every unit increase in PRS, the QTc interval prolonged by 4.0 ms (95% CI: [2.0, 6.1]; p-value: <0.001) in the additive model and 9.4 ms in the recessive model (95% CI: [4.6, 14.1]; p-value: <0.001). Serum ALT showed a modification effect on PRS-QTc prolongation under the recessive model. In the normal ALT group, each PRS unit increased QTc interval by 11.7 ms (95% CI: [6.3, 17.1]; p-value: 2.60E-5), whereas this effect was not observed in the elevated ALT group (0.9 ms; 95% CI: [-7.0, 8.8]; p-value: 0.823). CONCLUSION: Several candidate genetic variants are associated with QTc interval prolongation in SCD patients, and serum ALT acts as a modifying factor. The association of a CPS1 gene variant in both QTc and JTc duration adds to NOS1AP as evidence of involvement of the urea cycle and nitric oxide metabolism in cardiac repolarization in SCD. Larger replication studies are needed to confirm these findings and elucidate the underlying mechanisms.

Characteristics and outcomes of heart failure-related intensive care unit admissions in children with cardiomyopathy.

OBJECTIVE: The purpose of this study was to describe patient characteristics and outcomes of heart failure (HF)-related intensive care unit (ICU) hospitalizations in children with cardiomyopathy (CM). METHODS AND RESULTS: A query of the Pediatric Health Information System database, a large administrative and billing database of 43 tertiary children's hospitals, was performed. A total of 17,309 HF-related ICU hospitalizations from 2005 to 2010 of 14,985 children ≤18 years old were analyzed. Of those, 2,058 (12%) hospitalizations for CM-HF in 1,599 (11%) children were identified. Classification into CM subtypes was not possible owing to database limitations. The number of yearly CM-HF hospitalizations significantly increased during the study period (P = .036). Overall mortality was 11%, and cardiac transplantation occurred in 20% of hospitalizations. Mechanical circulatory support (MCS) was used in 261 (13%) of hospitalizations. Renal failure, MCS, respiratory failure, sepsis, and vasoactive medications were associated with mortality on multivariable analysis. Significant comorbidities associated with these hospitalizations included arrhythmias in 42%, renal failure in 13%, cerebrovascular disease in 6%, and hepatic impairment in 5%. CONCLUSIONS: HF-related ICU hospitalizations in children with cardiomyopathy are increasing. These children are at high risk for poor outcomes with an in-hospital mortality of 11%.

Cardiopulmonary resuscitation in hospitalized children with cardiovascular disease: estimated prevalence and outcomes from the kids' inpatient database.

OBJECTIVE: Hospitalized children with cardiovascular disease may be at increased risk of cardiac arrest; however, little data exist regarding prevalence, risk factors, or outcomes of cardiopulmonary resuscitation in these patients. We sought to characterize national estimates of cardiopulmonary resuscitation and death after cardiopulmonary resuscitation for hospitalized children with cardiovascular disease. SETTING: A total of 3,739 hospitals in 38 states participating in Kids' Inpatient Database. DESIGN: Retrospective analysis of the 2000, 2003, and 2006 Healthcare Cost and Utilization Project Kids' Inpatient Database was performed. Sample weighting was employed to produce national estimates. MEASUREMENTS AND MAIN RESULTS: Cardiovascular disease was identified in 2.2% of the estimated 22,175,468 (95% confidence interval 21,391,343-22,959,592) hospitalizations. Cardiopulmonary resuscitation occurred in 0.74% (3,698; 95% confidence interval 3,205-4,191) of hospitalizations of children with cardiovascular disease, compared with 0.05% (11,726; 95% confidence interval 10,647-12,805) without cardiovascular disease (odds ratio 13.8, 95% confidence interval 12.8-15.0). The highest frequency of cardiopulmonary resuscitation occurred with myocarditis (3.0% of admissions), heart failure (2.0%), and coronary pathology (2.0%). Compared with other forms of cardiovascular disease identified in this study, single-ventricle patients were the only subgroup who exhibited a higher mortality after cardiopulmonary resuscitation (mortality 65% vs. 55%; odds ratio 1.7 [95% confidence interval 1.2-2.6]), while those who had undergone cardiac surgery exhibited a lower mortality rate (mortality 48% vs. 57%; odds ratio 0.6 [95% confidence interval 0.5-0.8]). CONCLUSIONS: Cardiopulmonary resuscitation occurs in approximately 7 per 1,000 hospitalizations of children with cardiovascular disease, a rate greater than ten-fold that observed in hospitalizations of children without cardiovascular disease. Single-ventricle patients demonstrated increased mortality after cardiopulmonary resuscitation, while recent cardiac surgery was associated with a reduced odds of death after cardiopulmonary resuscitation. Further studies are needed to confirm these findings and develop techniques to prevent cardiac arrest in this high-risk population.

Response to intravenous potassium chloride supplementation in pediatric cardiac intensive care patients.

Potassium chloride (KCl) supplementation is common among critically ill children. Intravenous (IV) KCl supplementation for pediatric patients is poorly characterized. This study aimed to examine the efficacy and safety of IV KCL and to determine factors affecting patient responses to IV KCL in the pediatric cardiac intensive care unit (CICU). A retrospective review of 211 children (794 KCl doses) undergoing cardiac surgery or a hospital stay for heart failure in the CICU of a tertiary care teaching and referral children's hospital in 2011 was performed. Demographic data, weight, height, creatinine, and concomitant medications during each KCl dose were recorded and analyzed. Body surface area (BSA), creatinine clearance, and change in [K(+)] were calculated. The median age of the children was 4 months (range, 10 days-18 years). In this study, 151 KCl doses were administered to neonates (19 %), 307 doses (39 %) to females, and 510 doses (64 %) to patients with a BSA smaller than 0.33 m(2) (a group with relative renal insufficiency). The mean KCl dose was 0.97 ± 0.006 mEq/kg. No adverse events were associated with IV KCl administration. Blood/plasma [K(+)] increased 0.8 ± 0.02 mEq/L. The responses to KCl did not differ significantly between males and females, between neonates and children, or between patients with a BSA smaller than 0.33 m(2) and those with a BSA of 0.33 m(2) or larger. The responses to IV KCl were attenuated by concomitant furosemide (p = 0.01), amphotericin B (p < 0.01), and KCl in parenteral nutrition (p < 0.01). The responses were augmented by concomitant enalapril (p = 0.03), ethacrynic acid (p < 0.001), and hemodialysis (p < 0.01). Intravenous KCl can be administered safely for CICU patients. Responses to KCl are altered when it is given with certain medications. Intravenous KCl should be used cautiously in children receiving angiotensin-converting enzyme inhibitors. Future studies are needed for further characterization of factors affecting responses to IV KCl in children.

Characterization of extracorporeal membrane oxygenation for pediatric cardiac arrest in the United States: analysis of the kids' inpatient database.

To characterize the overall use, cost, and outcomes of extracorporeal membrane oxygenation (ECMO) as an adjunct to cardiopulmonary resuscitation (CPR) among hospitalized infants and children in the United States, retrospective analysis of the 2000, 2003, and 2006 Kids' Inpatient Database (KID) was performed. All CPR episodes were identified; E-CPR was defined as ECMO used on the same day as CPR. Channeling bias was decreased by developing propensity scores representing the likelihood of requiring E-CPR. Univariable, multivariable, and propensity-matched analyses were performed to characterize the influence of E-CPR on survival. There were 8.6 million pediatric hospitalizations and 9,000 CPR events identified in the database. ECMO was used in 82 (0.9 %) of the CPR events. Median hospital charges for E-CPR survivors were $310,824 [interquartile range (IQR) 263,344-477,239] compared with $147,817 (IQR 62,943-317,553) for propensity-matched conventional CPR (C-CPR) survivors. Median LOS for E-CPR survivors (31 days) was considerably greater than that of propensity-matched C-CPR survivors (18 days). Unadjusted E-CPR mortality was higher relative to C-CPR (65.9 vs. 50.9 %; OR 1.9, 95 % confidence interval 1.2-2.9). Neither multivariable analysis nor propensity-matched analysis identified a significant difference in survival between groups. E-CPR is infrequently used for pediatric in-hospital cardiac arrest. Median LOS and charges are considerably greater for E-CPR survivors with C-CPR survivors. In this retrospective administrative database analysis, E-CPR did not significantly influence survival. Further study is needed to improve outcomes and to identify patients most likely to benefit from this resource-intensive therapy.

Clinical and Laboratory Correlates of QTc Duration in Adult and Pediatric Sickle Cell Disease.

Background: Sickle cell disease, a common genetic disorder in African Americans, manifests an increased risk of sudden death, the basis of which is incompletely understood. Prolongation of heart rate-corrected QT (QTc) interval on the electrocardiogram, a standard clinical measure of cardiac repolarization, may contribute to sudden death by predisposing to torsades de pointes ventricular tachycardia. Methods: We established a cohort study of 293 adult and 121 pediatric sickle cell disease patients drawn from the same geographic region as the Jackson Heart Study (JHS) cohort, in which significant correlates of QT duration have been characterized and quantitatively modeled. Herein, we establish clinical and laboratory correlates of QTc duration in our cohort using stepwise multivariate linear regression analysis. We then compared our adult sickle cell disease data to effect-size predictions from the published JHS statistical model of QT interval duration. Results: In adult sickle cell disease, gender, diuretic use, QRS duration, serum ALT levels, anion gap, and diastolic blood pressure show positive correlation; hemoglobin levels show inverse correlation; in pediatric sickle cell disease, age, hemoglobin levels, and serum bicarbonate and creatinine levels show inverse correlation. The mean QTc in our adult sickle cell disease cohort is 7.8 milliseconds longer than in the JHS cohort, even though the JHS statistical model predicts that the mean QTc in our cohort should be > 11 milliseconds shorter than in the much older JHS cohort, a differential of > 18 milliseconds. Conclusion: Sickle cell disease patients have substantial QTc prolongation relative to their age, driven by factors some overlapping, in adult and pediatric sickle cell disease, and distinct from those that have been defined in the general African American community.

Prevalence and outcomes of pediatric in-hospital cardiopulmonary resuscitation in the United States: an analysis of the Kids' Inpatient Database*.

OBJECTIVE: Population-based data on pediatric in-hospital cardiopulmonary resuscitation in the United States are scarce. Single-center studies and voluntary registries may skew the estimated prevalence and outcomes. This study aimed to determine the prevalence and outcomes of pediatric cardiopulmonary resuscitation on a national scale. DESIGN: A retrospective analysis of the Healthcare Cost and Utilization Project 2006 Kids' Inpatient Database was performed. Sample weighting was employed to produce national estimates. SETTING: Three thousand seven hundred thirty-nine hospitals in 38 states participating with the Kids' Inpatient Database. PATIENTS: All patients <20 yrs of age hospitalized in participating institutions in 2006. MEASUREMENTS AND MAIN RESULTS: Cardiopulmonary resuscitation was performed in 5,807 (95% confidence interval 5259-6355) children with prevalence of 0.77 per 1,000 admissions. Most patients (68%) were <1 yr old, and 44% were female. On multivariable analysis, cardiopulmonary resuscitation was associated with respiratory failure (odds ratio 41.5, 95% confidence interval 35.4-48.8), myocarditis (odds ratio 36.6, 95% confidence interval 21.9-61.0), acute renal failure (odds ratio 21.6, 95% confidence interval 17.5-26.7), heart failure (odds ratio 3.8, 95% confidence interval 3.0-4.8), and cardiomyopathy (odds ratio 3.8, 95% confidence interval 3.2-4.7). Overall mortality was 51.8% and greater among patients ≥1 yr (68%) vs. <1 yr (44%) (odds ratio 2.7, 95% confidence interval 2.3-3.2). Factors associated with mortality among patients receiving cardiopulmonary resuscitation on multivariable analysis included acute renal failure (odds ratio 1.5, 95% confidence interval 1.1-1.9), hepatic insufficiency (odds ratio 1.5, 95% confidence interval 1.01-2.4), sepsis (odds ratio 1.2, 95% confidence interval 1.01-1.4), and congenital heart disease (odds ratio 1.2, 95% confidence interval 1.01-1.5). CONCLUSIONS: Cardiopulmonary resuscitation is performed in approximately one in 1,300 pediatric hospitalizations. Approximately half of patients receiving cardiopulmonary resuscitation do not survive to discharge. Independent risk factors for mortality after receiving cardiopulmonary resuscitation included congenital heart disease, age ≥1 yr, acute renal failure, hepatic insufficiency, and sepsis.

Neonatal midaortic syndrome and renal artery atresia presenting as malignant hypertension.

We report a case of congenital midaortic syndrome with bilateral renal artery atresia in a premature female neonate born by way of caesarean section secondary to acute onset of decreased fetal movement and polyhydramnios. The infant required cardiopulmonary resuscitation at birth, and initial echocardiogram exhibited normal intracardiac anatomy and mildly depressed left-ventricular systolic function. Within 24 h, the neonate developed severe systemic arterial hypertension and acute renal failure. Ultrasound demonstrated hyperechoic kidneys and a hypoplastic abdominal aorta. Angiography revealed severe suprarenal hypoplasia of the abdominal aorta with bilateral renal artery atresia. Medical support was withdrawn, and the patient died shortly thereafter.

Warfarin anticoagulation after congenital heart surgery at a large children's hospital.

Management of warfarin in pediatric patients remains a clinical challenge. Warfarin may be administered after congenital heart surgery, and the risks of morbidity can be high. Currently, no data exist to describe the initiation of warfarin and the risk factors for morbidity in post-congenital heart surgery patients. This study aimed to characterize the time required to reach anticoagulation for patients administered warfarin therapy after cardiac surgery and to identify and characterize the risk factors for supratherapeutic anticoagulation and adverse events after warfarin initiation. This retrospective study reviewed all patients between 2006 and 2011 who received warfarin anticoagulation after cardiac surgery at our institution. Factors associated with a prolonged time required to reach an international normalized ratio (INR) of 2 and factors related to supratherapeutic anticoagulation (INR ≥ 4) were identified. The inclusion criteria were met by 59 patients. The median time required to reach an INR of at least 2 after initiation of warfarin was 2 days (interquartile range (IQR), 2-4). The only groups that required a significantly longer time to reach an INR of 2 were those with a postoperative delay in initiation of warfarin and those receiving heparin anticoagulation before and during warfarin initiation. Nine patients experienced an INR of 4 or more. However, no thrombotic events occurred, and significant bleeding was uncommon. In the largest reported group of patients undergoing anticoagulation after cardiac surgery, warfarin was well tolerated across all age groups. The median time required to reach an INR of 2 after loading with warfarin was 2 days, and adverse events were uncommon.

Pediatric Extracorporeal Membrane Oxygenation Anticoagulation Protocol Associated with a Decrease in Complications.

Extracorporeal membrane oxygenation (ECMO) in pediatrics has rapidly progressed in recent years; however, there continues to be considerable variation in anticoagulation practices. In 2016, we implemented a standardized anticoagulation protocol in effort to reduce clotting and bleeding complications. A single-center retrospective analysis of pediatric patients requiring ECMO between 2014 and 2018 was performed. One hundred one ECMO cases in 94 pediatric patients met eligibility criteria (preprotocol = 64 cases; postprotocol = 37 cases). Demographics, ECMO parameters, complications, laboratories, and blood product requirements were analyzed for differences between the two groups. There was a significant decrease in the incidence of hematologic (p < 0.022), neurologic (p < 0.001), and renal complications (p < 0.001) in the postprotocol era. Incidence of bleeding, cardiac/pulmonary complications, and circuit changes were similar between the groups. The postprotocol group required fewer transfusions of red blood cells and cryoprecipitate. Additionally, platelet counts and fibrinogen levels were maintained higher in the postprotocol era. In conclusion, implementation of a standardized anticoagulation protocol was associated with improved anticoagulation parameters and a decrease in hematologic and neurologic complications, coagulopathy, renal injury, and blood product administration. We attribute these findings to transitioning to anti-Xa as a measure of heparinization and maintaining higher platelet counts.

High-dose sotalol is safe and effective in neonates and infants with refractory supraventricular tachyarrhythmias.

Our objective was to assess the efficacy and safety of high-dose sotalol in neonates and infants with refractory supraventricular tachycardia (SVT). SVT in neonates and infants can be refractory to primary therapies; therefore, secondary agents, e.g., sotalol, are often required to obtain control of SVT. Age-factor nomogram dosing of sotalol is widely used; however, our institution uses greater doses based on body surface area (approximately 150-200 mg/m(2)/d). A retrospective review of 78 inpatients receiving sotalol, after failing another antiarrthymic medication, at our institution from 2001 to 2008 was performed. Corrected QT intervals (QTc), 24-h Holter-monitoring results, and outpatient records were reviewed to assess safety and efficacy for patients ≤ 2 years of age. Median patient age at the time of initiation of therapy was 24 days (range 3-728). Forty-eight patients (62%) were neonates, and 36 (46%) had congenital heart disease. The median sotalol dosage was 152 mg/m(2)/day (range 65-244). The SVT of 70 patients (90%) was controlled with sotalol. No patients experienced significant QTc prolongation or proarrhythmia. Mean duration of follow-up was 3.3 ± 0.24 years. High-dose sotalol allows for safe and rapid control of refractory tachyarrhythmias in this young age group.