Leadless Pacemaker Implantation in Fontan Patients with Multimodality Imaging: Tips and Tricks.

Current leadless pacemaker (LP) systems, which have been developed and used in patients with normal cardiac anatomy, are rare and technically even more challenging to implant in patients with congenital heart diseases, especially with univentricular physiology and Fontan palliation. We report two cases of percutaneous LP implantation in an adult and a child, respectively, highlighting the unconventional approaches, different challenges, and use of multimodality imaging in patients who underwent a Fontan operation.

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Advanced imaging improves detection of baffle leaks and stenoses after atrial switch compared with transthoracic echocardiography.

Current guidelines for adults with atrial switch repair recommend baseline cardiovascular magnetic resonance (CMR) for assessment of ventricular size and function, systemic and venous baffle obstruction and leaks, and valvular function. It also recommends transthoracic echocardiography (TTE) for outpatient follow up. Many such patients with implanted cardiac devices may need cardiac computed tomography (CCT) when CMR is not feasible. This study reviews and compares CMR, transesophageal echocardiography (TEE), CCT, cardiac catheterization with angiography and TTE in detection of baffle problems in patients after atrial switch operation. The medical records of patients who had at least one imaging study performed after atrial switch operation at our center from 2010 to 2020 were retrospectively reviewed. Results are reported as descriptive statistics for demographics and imaging findings. The principal outcome measure was detection of baffle leak and/or baffle stenosis. Fifty-seven patients had at least one cardiac imaging study after atrial switch operation (36 Senning and 21 Mustard operations) during the study period. Nearly 33% (19/57) had baffle complications of stenosis and/or baffle leaks identified. All 57 patients had TTE performed but baffle problems were noted by TTE in only 8 (14%) patients (7 baffle stenosis and 1 baffle leak). Of the 49 patients without known baffle problems by TTE, 24 had advanced imaging (TEE/CCT/CMR/angiography). Advanced imaging identified baffle problems in nearly half (11/24, 46%) of them (7 baffle leaks and 4 baffle stenosis). Baffle problems were present in (8/23) patients with transvenous cardiac devices. Baffle complications are common after atrial switch operations and in our study occur in 1/3rd of the patients. However, TTE is not sensitive enough to recognize these complications. Advanced imaging for detection of baffle complications should be considered in all patients after atrial switch operation.

Seizures, Meds, and Vtach: A Journey to a Brugada Diagnosis.

ABSTRACT: Sudden cardiac arrest of cardiac etiology is rare in children and adolescents and most often occurs with exertion. Conversely, syncope is a common pediatric emergency department complaint but rarely is associated with a serious underlying cardiac disorder. This report describes a case of the channelopathy Brugada syndrome (BrS) as a cause of sudden cardiac arrest in a febrile preadolescent child taking medications known to affect cardiac conduction. The patient received cardiopulmonary resuscitation and was successfully defibrillated. Initial electrocardiogram (ECG) demonstrated findings consistent with BrS. Confirmatory electrophysiologic testing was performed, and an implantable cardiac defibrillator was placed. Pediatric emergency specialists must recognize both the importance of ECG in the workup of syncope and be familiar with the specific ECG findings suggestive of BrS. Ventricular arrhythmias that occur at rest should raise the suspicion of this genetic cardiac channelopathy, regardless of age.

Late transcatheter recanalization of a closed ductus arteriosus in a 2-month-old infant with tetralogy of Fallot and isolated left pulmonary artery.

Ductal stenting is increasing as an alternative to surgical shunts in cyanotic newborns. However, most reports include newborns with patent ducts who are often on prostaglandin before the procedure. We report a successful late transcatheter recanalization of the closed ductus arteriosus in a 2-month-old infant with tetralogy of Fallot, right aortic arch, and an isolated left pulmonary artery (LPA). We achieved adequate LPA growth before complete repair. There were no procedural complications. This procedure is rare in Western countries due to universal pulse oximetry screening and increased fetal diagnoses. The procedure is technically challenging in the absence of any angiographic flow into the ductus. Evidence of a ductal ampulla increases the chances of success.

Pre-excitation induced ventricular dysfunction and successful berlin heart explantation after accessory pathway ablation.

A 13 kg, 20 month-old, Caucasian girl, presented with cardiomyopathy, biventricular dysfunction and pre-excitation on electrocardiogram. She had normal intracardiac anatomy with severely dilated left ventricle and severely diminished biventricular function (Fig. 1). She was treated with milrinone and epinephrine infusions, mechanical ventilation and listed for heart transplant. She underwent Berlin Heart EXCOR biventricular assist device (BiVAD) placement (30 ml LVAD and 25 ml RVAD pumps). No supraventricular tachycardia (SVT) was inducible or noted during her hospitalization. First ablation attempt without BiVAD support was unsuccessful; however, 18 days post BiVAD implantation, another electrophysiology study and successful radiofrequency ablation of a right anterolateral accessory pathway was performed on BiVAD support. After successful ablation and loss of pre-excitation, the cardiac dysfunction rapidly improved with initial improvement noted as early as 48 h after the successful ablation. Due to recovery of cardiac function, a BiVAD wean protocol was initiated and BiVAD explantation was performed 48 days after the implant (30 days after the successful ablation). To the best of our knowledge, this is the first report of successful BiVAD explantation.

Primary cerebellopontine angle glioblastoma in an adult.

Supratentorial glioblastoma is the most frequent primary intracranial tumor in adults. Its localization in the cerebellum is extremely rare and such tumor presenting as cerebellopontine angle (CPA) mass in an adult is even rarer. We report the case of primary cerebellar glioblastoma in a 52-year-old woman who presented clinically and radiologically as CPA mass. Its clinical presentation, radiological findings, and treatment instituted are discussed in view of pertinent literature.

Malignant peripheral nerve sheath tumor presenting as orbito temporal lump: Case report and review of literature.

Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma. The most common anatomical sites include the upper and lower extremities and trunk and less commonly the head and neck. To our knowledge, few patients with a cranial or facial MPNST have been reported. We report such a lesion in a 35-year-old woman who presented with left sided rapidly progressive proptosis and visual loss due to an orbital lump extending up to the temporal lobe. Cranial imaging showed a huge mass invading the orbital wall and temporal bone. The presumptive diagnosis was a malignant orbital tumor. Preoperative fine needle aspiration cytology of the orbital mass came to be neurofibroma. Near total resection of the tumor was done. Histopathology revealed MPNST which was subsequently confirmed on the basis of immunopositivity for S-100. The patient recovered uneventfully and was discharged 8 days after surgery with an advice to attend cancer institute for possible radiotherapy.

Supratentorial extradural hematoma in children: An institutional clinical experience of 65 cases.

AIM AND OBJECTIVE: To survey the epidemiology, management, and severity of extradural hematoma (EDH) in children. MATERIALS AND METHODS: All patients of EDH (n = 65) in the age group of 0-16 years admitted to our department during the period of August 13 and July 14 were analyzed retrospectively from the hospital records. In all patients, age, sex, mode of injury, clinical presentation, site of EDH, management, duration of hospitalization, and outcome were evaluated. OBSERVATION AND RESULTS: Of 65 patients, males were 70.76% with a male to female ratio of 2.4:1, most of the victims (47.69%) were in the age group of 11-16 years. Mean duration of hospitalization was 4.32 days. The most common mode of injury was fall from height in 29 cases (44.61%) followed by road traffic accident (RTA) in 23 cases (35.35%). Temporoparietal EDH was the most common computed tomography finding present in 22 (33.84%) patients. 67.69% patients presented to casualty with minor head injury having Glasgow coma scale (GCS) between 14 and 15. Most common presenting feature was vomiting in 52 cases (80%) and next to it was altered sensorium. The mortality rate was 7.69% (n = 5). CONCLUSION: Extradural hematoma is a life-threatening entity encountered in pediatric head injury. Timely intervention and diagnosis decrease mortality to a great degree. Most of the mortality is encountered in patients who presented late at the neurosurgical unit with low GCS.

Management of superior sagittal sinus injury encountered in traumatic head injury patients: Analysis of 15 cases.

BACKGROUND: Significant dural sinus injury occurs in 1.5- 5% of all head injuries and injury to superior sagittal sinus accounts for 70-80% of these. Its management is also a challenging issue to every neurosurgeon. MATERIALS AND METHODS: In a period of one year (Aug 2011 to july2012) out of 549 cases of head injuries operated in our department analysis of 15 cases (2.73%) shown to have superior sagittal sinus injury. Three dimensional reconstruction of skull bone during CT scan helped us to diagnose possible dural sinus injury pre-operatively. RESULTS: SSS injury was due to direct impact of fracture segment (5 cases), extension of fracture line over sinus area (4 cases) or due to coronal suture diastases (6 cases). Injury to anterior third of sagittal sinus occurred in 12 cases (80%) and injury to middle third occurred in 3 cases (20%). Small tear over superior sagittal sinus was found in 7 cases (46.66%) and was managed with Gelfoam(®) compression only and in two cases (13.33%) were large tear, which was managed with sinoraphy. In four cases (36.34%) hitch stitches over Gelfoam(®) to adjacent bone given and in rest two cases (13.33%) a strip of bone was left over the sinus area and bilateral hitch stitches were applied. CONCLUSION: Injury to SSS in traumatic head injury patient though rarely encountered during surgery, the management is challenging to every neurosurgeon. Preoperative suspicion and combination of surgical techniques rather than one single technique may be effective in decreasing the sinus related mortality rate.

Primary malignant peripheral nerve sheath tumor at unusual location.

Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma. Most arise in association with major nerve trunks. Their most common anatomical sites are the proximal portions of the upper and lower extremities and the trunk. MPNSTs have rarely been reported in literature to occur in other unusual body parts. We review all such cases reported till now in terms of site of origin, surgical treatment, adjuvant therapy and outcome and shortly describe our experience with two of these cases. Both of our case presented with lump at unusual sites resembling neurofibroma, one at orbitotemporal area and other in the paraspinal region with characteristic feature of neurofibroma with the exception that both had very short history of progression. They underwent gross total removal of the tumor with adjuvant radiotherapy postoperatively. At 6-month follow-up both are doing well with no evidence of recurrence.

Hemangiopericytoma of neck extending to craniovertebral junction treated by surgery, pre- and postoperative radiotherapy.

BACKGROUND: Hemangiopericytoma (HPC) is a rare tumor of uncertain malignant potential arising from mesenchymal cells with pericytic differentiation. It accounts for 3-5% of soft tissue sarcomas, and 1% of vascular tumors. The treatment of choice is a primary wide surgical resection with adjuvant radiotherapy (RT) reserved for cases of incomplete removal. CASE DESCRIPTION: We report a case of a 24-year-old female with a rapidly growing, highly vascular swelling in nape of the neck extending deep into the craniovertebral (CV) junction accompanied by extradural/intraspinal, and intracranial involvement. An incisional biopsy revealed a cellular, highly vascular tumor with HPC-like features. The patient received preoperative RT, which reduced both the size and vascularity of the lesion, facilitating subsequent near complete resection. Further postoperative RT resulted in a good clinical outcome, with no tumor recurrence observed at 2 postoperative years. CONCLUSION: HPC of the soft tissues of neck accompanied by deep extension to the CV junction is uncommon. A high index of suspicion is required to diagnose these cases. which may be treated with preoperative RT (to reduce the lesion size/vascularity), aggressive surgical resection, followed by postoperative adjunctive radiation treatment as well.

Intracerebral pial arteriovenous fistula with large venous varix: A rare case report.

BACKGROUND: Intracerebral pial arteriovenous fistulas (AVFs) are rare vascular lesions of the brain. These lesions are composed of one or more direct arterial connection to a single venous channel without true intervening nidus and usually have associated venous varix or giant venous aneurysms. Intracerebral varices are occasionally associated with high-flow AVF, and usually treated by interrupting the feeding arteries leaving the varices intact. CASE DESCRIPTION: We report a rare case of a 24-year-old male with a single-channel pial AVF of the left cerebral hemisphere, which was fed by the left anterior cerebral artery (ACA) and was associated with large venous varix and continuous varicose venous dilatation. This superficially located varix was over 6 cm in diameter posing significant mass effect and had calcified walls. Direct surgical flow disconnection followed by removal of large varix resulted in complete disappearance of pial AVF without complication. CONCLUSION: Though endovascular occlusion of feeding arteries offers a simple and safe option, direct surgical removal should be considered in rare cases of intracerebral superficially located large AVF with calcified wall and mass effect.

Junctional ectopic tachycardia after congenital heart surgery in the current surgical era.

To determine the incidence of postoperative junctional ectopic tachycardia (JET) in a modern cohort of pediatric patients, evaluate possible risk factors for JET, and examine the effects of JET on postoperative morbidity and mortality. JET is common after congenital heart surgery. JET-related mortality has been a rare event at our center, which is different from previous reports. We reviewed records for pediatric patients who had postoperative arrhythmias between January 2006 and June 2010 at a large tertiary-care children's hospital. We performed a matched case-control study to identify risk factors for JET and a matched-cohort study to compare outcomes between patients and controls. Whenever possible, each JET case was randomly matched to two controls on the basis of lesion, repair, and surgical period. We identified 54 patients with JET (incidence = 1.4 %). After multivariate logistic regression analysis, low operative weight, cardiopulmonary bypass (CPB) duration >100 min, and immediate postoperative serum lactic acid level >20 mg/dl were associated with increased odds of developing JET. Patients with JET had longer mechanical ventilation time, cardiac intensive care unit (CICU) stay, and hospital stay. There was only one death in JET group (1.8 %) with no significant difference compared with the control group. JET remains a relatively common postoperative arrhythmia, but it is less frequent than previously reported. JET occurs more commonly in smaller patients with longer CPB runs and significant postoperative lactic acidosis levels. Mortality associated with JET is lower than historically reported, but morbidity remains high.

B-type natriuretic peptide in the recognition of critical congenital heart disease in the newborn infant.

OBJECTIVE: Infants with congenital heart disease having obstruction to the left ventricular outflow and ductal-dependent systemic circulation can present critically ill with shock. Prompt disease recognition and initiation of prostaglandins are necessary to prevent excess morbidity and mortality. We assessed a large cohort of newborn infants with ductal-dependent systemic circulation to determine if B-type natriuretic peptide (BNP) is consistently elevated at presentation, potentially aiding in diagnosis and treatment. METHODS: The clinical records of infants with left-sided obstructive lesions admitted from January 2005 to June 2009 were reviewed. Infants were divided into 2 groups: group 1 was diagnosed with cardiogenic/circulatory shock at presentation, and group 2 consisted of infants with ductal-dependent systemic circulation without evidence of shock. B-type natriuretic peptide levels and other variables between the groups were compared. RESULTS: All patients with critical congenital heart disease presenting with shock had elevated BNP levels, ranging from 553 to greater than 5000 pg/mL. Infants in group 1 (shock, n = 36) had significantly higher median BNP levels of 4100 pg/mL at presentation compared with group 2 patients (no shock, n = 86), who had a median BNP of 656 pg/mL (range, 30-3930 pg/mL; P < 0.001). Every 100 U of increase in BNP at presentation increased the likelihood of shock (odds ratio, 100; P < 0.001). CONCLUSIONS: B-type natriuretic peptide is markedly elevated in neonates presenting in shock secondary to left-sided obstructive heart disease and is an important diagnostic tool to aid in the rapid identification and treatment of these patients.