Sudden cardiac death under anesthesia in pediatric patient with Williams syndrome: a case report and review of literature.

Williams syndrome is a complex syndrome characterized by developmental abnormalities, craniofacial dysmorphic features, and cardiac anomalies. Sudden death has been described as a very common complication associated with anesthesia, surgery, and procedures in this population. Anatomical abnormalities associated with the heart pre-dispose these individuals to sudden death. In addition to a sudden and rapid downhill course, lack of response to resuscitation is another significant feature seen in these patients. The authors report a five-year-old male with Williams syndrome, hypothyroidism, and attention deficit hyperactivity disorder. He suffered an anaphylactic reaction during CT imaging with contrast. Resuscitation was unsuccessful. Previous reports regarding the anesthetic management of patients with Williams are reviewed and the potential for sudden death or peri-procedure related cardiac arrest discussed in this report. The authors also review reasons for refractoriness to defined resuscitation guidelines in this patient population.

Moyamoya disease with peripheral pulmonary artery stenoses and coronary artery fistulae.

Moyamoya is a progressive disorder of the cerebral vasculature. Our report describes a rare case of Moyamoya disease with distal peripheral pulmonary artery stenoses and coronary fistulae in a 12-year-old Caucasian female patient.

Carotid artery stent migration in a child with Williams syndrome.

We report a case of distal migration of a stent from the brachiocephalic artery to the distal right common carotid artery 7 months after implantation in a 5-year-old child with Williams syndrome. There were no neurological sequelae and the migrated stent remained widely patent 5 years following implantation.

Preliminary experience with a combination of dexmedetomidine and propofol infusions for diagnostic cardiac catheterization in children.

No specific regimen has been universally accepted as ideal for procedural sedation during cardiac catheterization in infants and children. In this paper, we retrospectively describe our preliminary experience with a continuous infusion of dexmedetomidine and propofol for sedation during cardiac catheterization in children with congenital heart disease. The short-half life of these two drugs creates a potential for easier titration, quicker recovery and less prolonged sedation-related adverse effects. This combination was not only able to limit the dose of either drugs, but was also very stable from cardio-respiratory standpoint. There were no adverse effects noted in our two patients. This initial experience showed that the combination of propofol and dexmedetomidine as a continuous infusion may be a suitable alternative for sedation in spontaneously breathing children undergoing cardiac catheterization.

Percutaneous transcatheter patent foramen ovale closure using the right internal jugular venous approach.

Percutaneous transcatheter closure of a patent foramen ovale (PFO) is a therapeutic option in patients with paradoxical embolism. For patients in whom PFO closure is indicated when a femoral venous approach is not possible, we describe the successful closure of two PFOs using the right internal jugular venous approach.

Compassionate use of the amplatzer ASD closure device for residual postinfarction ventricular septal rupture following surgical repair.

We report successful transcatheter closure of a post-MI ventricular septal rupture acutely following unsuccessful surgical repair. Catheter closure was accomplished by the use of a 26-mm Amplatzer atrial septal occluder. Initial attempts to close the defect with the use of 28-mm and 33-mm CARDIOSEAL were unsuccessful. Closure technique, immediate and long-term follow-up outcomes are reported.

Percutaneous transcatheter closure of patent foramen ovale in patients with paradoxical embolism.

BACKGROUND: Percutaneous transcatheter closure of patent foramen ovale (PFO) is used as an alternative to surgery or long-term anticoagulation for the treatment of patients with paradoxical embolism and PFO. METHODS AND RESULTS: We report the immediate and long-term clinical and echocardiographic outcome of 110 consecutive patients (58 males, mean age 47+/-14 years) who underwent transcatheter closure of PFO because of paradoxical embolism between 1995 and 2001. Procedural success, defined as successful deployment of the device and effective occlusion (no, or trivial, shunt after device placement), was achieved in all (100%) patients. There was no in-hospital mortality, 1 device migration requiring surgical intervention (0.9%), and 1 episode of cardiac tamponade (0.9%) requiring pericardiocentesis. A progressive increment in full occlusion was observed (44%, 51%, 66%, and 71% at 1 day, 6 months, and 1 and 2 years, respectively, after device placement). At a mean follow-up of 2.3 years, 2 patients experienced recurrent neurological events (1 fatal stroke and 1 transient ischemic attack), representing an annual risk of recurrence of 0.9%. In addition, 4 (3.6%) of the patients required reintervention for device malalignment or significant shunt. Kaplan-Meier analysis showed a freedom from recurrent embolic events and reintervention of 96% and 90% at 1 and 5 years, respectively. CONCLUSIONS: Transcatheter closure of PFO is a safe and effective therapy for patients with paradoxical embolism and PFO. It is associated with a high success rate, low incidence of hospital complications, and low frequency of recurrent systemic embolic events.