Aorta to left ventricle tunnel presenting with cardiac failure in a neonate.

We report an unusual case of an aorta to left ventricular communication in a neonate presenting with cardiac failure. Although extremely rare, this defect is the most common cause of abnormal blood flow from the aorta to a ventricle in infancy. Early diagnosis and prompt surgical repair are critical to management and results are generally excellent. Long-term follow-up is required to monitor for aortic insufficiency.

Adenosine sensitive left ventricular summit ventricular tachycardia in a pre-adolescent: case report.

Background: Sustained forms of tachycardia especially from the left ventricular summit are rare. Adenosine sensitive outflow tachycardias, especially from the left ventricular summit, are rarer still. These arrhythmias may be exercise or stress induced as they are facilitated by catecholamines and characteristically terminate with adenosine, vagal manoeuvres, and beta-blockers. The surface 12-lead electrocardiogram can be used to localize the anatomic site of origin before catheter ablation; however, prediction of the precise origin may still be challenging due to the intimate and complex anatomy of the outflow tracts. Case summary: A 12-year-old female presented to an emergency room with frequent runs of wide complex tachycardia that terminated with adenosine but would spontaneously reinitiate. After three additional temporary terminations with adenosine and because of an inability to completely eliminate tachycardia, she was started on an esmolol infusion that resulted in an abrupt termination of tachycardia. At follow-up, she reported breakthrough episodes of tachycardia with exercise, especially associated with beta-blocker non-compliance. The rest of her cardiac testing was normal apart from an anomalous right coronary artery origin from the left coronary sinus. Given the increased frequency of symptomatic palpitations and medication non-compliance, she underwent an electrophysiology study. During the study, a ventricular tachycardia was successfully mapped to an epicardial focus at the left ventricle summit and was successfully ablated. Discussion: The response of this patient's ventricular tachycardia to adenosine suggests a triggered mechanism. To our knowledge, this is the first unambiguous example of left ventricular tachycardia due to cAMP-mediated triggered activity in this age group.

Ventricular tachycardia in the setting of a large cardiac fibroma in a pediatric patient.

Ventricular tachycardia and cardiac tumors are both extremely rare diagnoses in pediatric patients. We report a pediatric case of cardiac fibroma that was noted during the work up of ventricular tachycardia in a young patient concomitantly diagnosed with severe acute respiratory syndrome coronavirus 2.

Sudden cardiac arrest as a presenting symptom of mitral valve prolapse.

Mitral valve prolapse (MVP) is a common and predominantly benign condition; however, occasional reports of cardiac arrest in individuals with MVP have raised concern for a malignant subtype. The risk of sudden cardiac arrest in MVP is twice as high as that in the general population. The exact aetiology and risk predictors are elusive, but identification is necessary, if we are to protect patients at risk for cardiac arrest. This report highlights cardiac arrest as the initial presenting symptom of MVP. Rapid initiation of bystander cardiopulmonary resuscitation and time to first shock are key predictors of prognosis. Better screening to identify individuals with malignant phenotypes may aid in reducing the morbidity and mortality in patients with a predisposition for life-threatening arrhythmias.

Case of WPW and anomalous right coronary in a teenager with cardiac symptoms.

A 17-year-old African-American man was being followed for palpitations and chest pain. CT angiography revealed an anomalous right coronary artery from the left coronary sinus and he underwent unroofing of the right coronary ostium. There was a manifest pre-excitation on postoperative ECGs, and review of prior ECGs at initial presentation showed subtle pre-excitation suggesting a left lateral pathway. An electrophysiology study revealed easily inducible supraventricular tachycardia (SVT) and rapid anterograde conduction via the pathway which was successfully ablated. Eight months postablation, the patient remains asymptomatic with no evidence of pre-excitation on ECG.

Malignant mitral valve prolapse: an uncommon variation of a common condition.

Mitral valve prolapse affects up to 3% of the population, with an annual risk of death of up to 2% a year. Presentation is often in the third to fourth decade of life. This report details an adolescent with mitral valve prolapse with non-specific symptoms but investigative findings of ventricular ectopy at higher heart rates. These patients warrant close surveillance to monitor for progressive arrhythmias and may progress to need an ICD.

Electrocardiogram Screening in Children with Congenital Sensorineural Hearing Loss: Prevalence and Follow-up of Abnormalities.

Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other. A board-certified pediatric cardiologist retrospectively determined the clinical significance of ECG changes. For follow-up analysis, children with heart disease, known syndromes, or inaccessible records were excluded. Results Among 772 children who had ECGs, 215 (27.8%) had abnormal results: 35 (4.5%) with QT abnormalities and 180 (23.3%) with other abnormalities. For children with QT abnormalities meeting inclusion criteria (n = 30), follow-up measures included cardiology referral (46.6%), repeat ECG by ear, nose, and throat (ENT) specialist (20%), clearance by ENT specialist with clinical correlation and/or comparison with old ECGs (20%), and pediatrician follow-up (6.7%). Documentation of further workup by ENT or referral was absent for 6.7%. For children with other ECG changes meeting inclusion criteria (n = 136), abnormalities were documented for 57 (41.9%); normal QT without other abnormality was documented for 18 (13.2%). The most common follow-up referrals were to pediatricians (16.9%) and cardiologists (10.3%). Among patients with clinically significant non-QT abnormalities mandating further evaluation (n = 122), 38 (31.1%) had documented follow-up in medical records. Conclusion There is a high prevalence of ECG abnormalities among children with congenital SNHL. If findings are confirmed by future studies, screening should be considered for congenital unilateral or bilateral SNHL, regardless of severity. We describe a standardized protocol for ECG screening/follow-up.

Early extubation in pediatric heart surgery across a spectrum of case complexity: Impact on hospital length of stay and chest tube days.

Early extubation is increasingly common in congenital heart surgery, but there are limited outcomes data across the spectrum of case complexity. We performed a retrospective review of 201 pediatric operations using cardiopulmonary bypass between 2012 and 2014. Patients extubated in the operating room or immediately on arrival to the ICU were compared to those extubated by traditional protocols. In-hospital mortality, major complications, need for re-intubation, hospital length of stay, and chest-tube days were compared between groups and by Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery Congenital Heart Surgery (STAT) mortality category. Outcome measures of hospital length of stay and chest tube days were analyzed using multivariable regression modeling. Early extubation subjects were older, weighed more, had shorter bypass and aortic cross-clamp time, more often received caudal anesthesia, and had shorter hospital length of stay and fewer chest tube days. Subjects not extubated early had more chromosomal abnormalities, more preoperative co-morbidities, and had more major complications. Inhospital death, major complications, and re-intubation were rare outcomes for both groups across all STAT categories. Multivariable regression analysis showed that cardiopulmonary bypass time was a significant predictor of hospital length of stay and chest tube days. Hospital LOS and chest tube days were significantly lower for the early extubation group in both the unadjusted and adjusted analyses. Early extubation can be performed safely in congenital heart surgery across a spectrum of case complexity. No increased early mortality or re-intubation was observed with early extubation although there were important differences between the groups that merits further study. The potential benefits of early extubation include decreased hospital length of stay and fewer chest tube days, particularly in young children and patients with long bypass times.

Improved Results of Aortic Arch Reconstruction in the Norwood Procedure.

BACKGROUND: Despite several surgical modifications, the reported incidence of neoaortic coarctation after stage 1 palliation (S1P) of hypoplastic left heart syndrome remains high. A modification of aortic arch reconstruction that may decrease the incidence of neoaortic coarctation after S1P is described. METHODS: Between January 2008 and May 2013, 114 patients with hypoplastic left heart syndrome underwent S1P. The hospital records of the 101 surviving patients (89%) were reviewed. Demographic data and perioperative variables for S1P were collected. A modified technique for aortic arch reconstruction that contains a bovine pericardial patch tailored and sutured in a specific way was used in 51 patients (group MT), whereas the traditional technique was used in 50 patients (group TT). Clinical echocardiographic and cardiac catheterization data were analyzed to determine the incidence and location of neoaortic coarctation. RESULTS: There were no significant differences in the baseline characteristics, the intraoperative course, or hospital mortality between the two groups of subjects. Duration of ventilation, intensive care unit stay, and hospitalization were significantly shorter in group MT. The mean follow-up was 2.02 ± 0.63 years and 3.98 ± 0.66 years in each group, respectively. Coarctation developed in 4 of 51 patients (7.8%) in group MT compared with 10/50 patients (20%) in group TT. Multivariable logistic regression analysis showed that the estimated odds of coarctation for group MT were 0.41 (95% confidence interval: 0.04, 4.32) times the estimated odds of coarctation for group TT. CONCLUSIONS: The described technique may decrease the incidence of recoarctation after S1P by minimizing aortic arch and descending aorta distortion and providing ample enlargement of the aorta at its narrowest diameter.

Is additional pulsatile pulmonary blood flow beneficial to patients with bidirectional Glenn?

OBJECTIVE: To compare the results of bidirectional Glenn when performed with or without pulsatile pulmonary blood flow in a cohort of patients with a single ventricle. METHODS: Records of 212 patients undergoing staged single ventricle palliation during a 10-year period were retrospectively reviewed. Of those, 103 (33 in pulsatile group A and 70 in nonpulsatile group B) were selected. RESULTS: Demographics and pre- and intraoperative variables were comparable for both groups. There was no difference in oxygen saturations immediately after the bidirectional Glenn in the 2 groups. The duration and output of chest tube drainage, incidence of chylothorax, and total length of stay was higher in group A. There was no difference in the number of diuretics or oxygen requirement upon discharge between groups. Pre-Glenn measurements showed a mean McGoon ratio in group A of 1.5 (1.46-1.57) and in group B of 1.59 (1.53-1.7) (P = .11); however, there was a significant difference in the ratio between groups at pre-Fontan measurements: group A, 1.76 (1.73-1.79) and group B, 1.6 (1.53-1.66) (P < .05). At pre-Fontan measurements there was a significant difference in mean pulmonary artery pressure between group A (14 mm [12.8-15.2]) and group B (10 mm [9.7-11]) (P < .05) and a trend toward higher incidence of venovenous collaterals in group A. There was no perioperative or interstage mortality in either group. CONCLUSIONS: Pulsatile bidirectional Glenn is associated with better pulmonary artery growth, which might improve long-term outcomes after Fontan. However, it was associated with a higher postoperative complication rate.

Transesophageal echocardiography in critically ill acute postoperative infants: comparison of AcuNav intracardiac echocardiographic and microTEE miniaturized transducers.

BACKGROUND: Multiple barriers to transthoracic echocardiography are present in critically ill infants immediately after surgery. Transesophageal echocardiography (TEE) is sometimes needed to obtain specific important information that transthoracic echocardiography fails to demonstrate. Formerly, the investigators used the AcuNav intracardiac echocardiographic (ICE) intravascular ultrasound transducer (8 Fr, 2.5 mm, 64-element crystal array, multifrequency [5.5-10 MHz], single longitudinal plane, linear phased array [Siemens Medical Solutions USA, Inc., Mountain View, CA]). Recently, the investigators have also used the microTEE transducer (8-mm transducer tip, 5.2-mm shaft, multifrequency [3-8 MHz], multiplane phased array, 32-element probe [Philips Medical Systems, Andover, MA]). Both transducers have two-dimensional, M-mode, color Doppler, and pulsed-wave and continuous-wave Doppler capabilities. The aim of this study was to compare the efficacy, safety, ease of insertion, capabilities, utilization, and cost of the AcuNav ICE transducer versus those of the microTEE transducer. METHODS: A retrospective review of all 50 postoperative critically ill infants who underwent TEE using the AcuNav and microTEE in the past 5 years was conducted. TEE was performed as ordered by the attending physician to answer a specific question not answered by transthoracic echocardiography. RESULTS: In all cases, the clinical information sought was obtained. The AcuNav ICE transducer was safe, easy to insert through the transnasal route, and did not require paralysis; however, it had a limited number of echocardiographic views and had greater sterilization cost. The microTEE transducer had greater echocardiographic capabilities and lower sterilization cost; however, it was slightly more difficult to insert, had a few manageable complications, and required more sedation and paralysis. CONCLUSIONS: TEE in this setting has increased because of demonstrated efficacy and safety. Both the AcuNav ICE and microTEE transducers are useful and effective in this critical clinical scenario.

Hodgkins disease in relapse presenting as bone tumor.

A 10-year-old male child presented with multiple lymph node swellings. A diagnosis of Hodgkins disease was made on histopathological examination. The patient developed relapse six months after his last chemotherapy as a solitary bone tumor, which is rare. Immunohistochemical evaluation helped for the correct typing of Hodgkins disease.