Michael de Moor

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.

Left main coronary artery compression syndrome: Evaluation with 64-slice cardiac multidetector computed tomography

A 28-year-old man presented to the emergency department with progressive shortness of breath complicated by large hemoptysis. At 3 months of age, he had been diagnosed with a “hole in his heart” in the Ukraine and had undergone 3 coronary catheterizations (2 in Russia, 1 in India; results unavailable). His parents had declined options for treatment. After stabilization in the emergency department, he was transferred to coronary care, where echocardiography demonstrated a persistent ductus arteriosus (PDA) measuring 0.9×2.0 cm with a right-to-left shunt consistent with Eisenmenger syndrome. Left ventricular function was severely impaired (ejection fraction=21%). Cardiac 64-slice multidetector computed tomography (MDCT) was undertaken to provide optimal depiction of the PDA and main pulmonary artery (PA) and confirmed a widely patent PDA measuring 2.0 cm in largest diameter (Figure …A 28-year-old man presented to the emergency department with progressive shortness of breath complicated by large hemoptysis. At 3 months of age, he had been diagnosed with a “hole in his heart” in the Ukraine and had undergone 3 coronary catheterizations (2 in Russia, 1 in India; results unavailable). His parents had declined options for treatment. After stabilization in the emergency department, he was transferred to coronary care, where echocardiography demonstrated a persistent ductus arteriosus (PDA) measuring 0.9×2.0 cm with a right-to-left shunt consistent with Eisenmenger syndrome. Left ventricular function was severely impaired (ejection fraction=21%). Cardiac 64-slice multidetector computed tomography (MDCT) was undertaken to provide optimal depiction of the PDA and main pulmonary artery (PA) and confirmed a widely patent PDA measuring 2.0 cm in largest diameter (Figure …

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. Cathet Cardiovasc Intervent 2003;59:230–233.

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. Catheter Cardiovasc Interv 2003;60:536–539.

Dynamic Left Main Coronary Artery Obstruction Associated With Williams Syndrome

A 5-year-old boy with Williams syndrome (WS) presented with a history of diffuse recurrent obstructive vascular disease. At the age of 12 months, he underwent pericardial patch augmentation of the ascending aorta and pulmonary arteries. He subsequently required Dacron patch repair of the ascending aorta and aortic arch at 3 years of age. In the interim, he had undergone percutaneous transluminal angioplasty and stenting for recurrent peripheral pulmonary artery stenoses and aortic recoarctation. On routine follow-up, he was found to have worsening right ventricular hypertension associated with disparate lung perfusion on a nuclear perfusion scan. He was brought to the catheterization laboratory for further investigation. Angiography showed recurrent pulmonary artery in-stent restenosis and aortic recoarctation. Coronary …

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.

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.

Myocardial infarction in a neonate with cyanotic congenital heart disease

A male neonate was referred to the Red Cross War Memorial Childrens Hospital, Cape Town, on the second day of life because of cyanosis . The pregnancy and delivery had been uncomplicated and his birth weight was 2770 g . He was moderately cyanosed but not distressed or in cardiac failure . Abnormalities were confined to the cardiovascular system and on auscultation there was a single second heart sound, an aortic ejection click, and a grade-2/6 ejection systolic murmur at the upper left sternal border. On chest radiograph, the lung fields were oligemic, the heart was slightly enlarged, and there was a pulmonary bay . The ECG (Fig. 1) showed a rate of 150/min, sinus rhythm, PR interval 0.10 s, axis -20° (left-axis deviation forage) . There were low anterior forces for age . The hemoglobin was 18 .9 g/dl, hematocrit 58%, and the platelet count 270,000/mm 3. Two-dimensional echocardiography confirmed the diagnosis of pulmonary atresia with intact ventricular septum and hypoplastic right ventricle . He was treated on oral prostaglandin E 2 , 125 μg, every hour to maintain ductal patency [7] and a palliative shunt procedure was planned at 2-3 weeks of age . However, ten days after admission, despite a continuous murmur indicating ductal patency, his condition deteriorated abruptly becoming tachypneic with signs of low-output cardiac failure . Cardiac catheterization was performed to carry out balloon atrial septostomy, and to assess the size of the pulmonary arteries and the right ventricle . Adequate-sized pulmonary arteries were shown to be perfused by the ductus arteriosus . The right ventricular cineangiogram showed the right ventricle to be bipar-

Improvement and Defervescence of Persistent Fever After One Course of Intravenous Immunoglobulin in a Patient With Bacterial Infective Endocarditis

Infective endocarditis (IE) is a microbial infection of the endocardial surface of the heart. Because of the increased survival rate of children with congenital heart disease and other risk factors for IE, such as indwelling vascular lines, IV drug use, invasive procedures, and immunosuppressive disorders, there has been an increase in the population at risk for IE [1]. Conventional management of IE involves prolonged treatment with antimicrobial agents to eliminate the invading organisms that are harbored within heart vegetations. When positive blood cultures persist despite proper medical regimens, surgical intervention is indicated to remove the infected vegetations [1]. At one time, peripheral manifestations of IE were considered to result from embolic phenomena. However, it is now well established that other mechanisms, most importantly immunologic mechanisms, are involved in the peripheral manifestations and pathogenesis of IE [2]. In this article, we present a case report of a patient with IE who had persistent fever despite adequate antimicrobial treatment and negative blood cultures, most likely as a consequence of an immune complex disease, and who had a dramatic improvement after one course of intravenous immunoglobulin (IVIg) therapy. Case Report

Left Main Coronary Artery Compression Syndrome

A 28-year-old man presented to the emergency department with progressive shortness of breath complicated by large hemoptysis. At 3 months of age, he had been diagnosed with a “hole in his heart” in the Ukraine and had undergone 3 coronary catheterizations (2 in Russia, 1 in India; results unavailable). His parents had declined options for treatment. After stabilization in the emergency department, he was transferred to coronary care, where echocardiography demonstrated a persistent ductus arteriosus (PDA) measuring 0.9×2.0 cm with a right-to-left shunt consistent with Eisenmenger syndrome. Left ventricular function was severely impaired (ejection fraction=21%). Cardiac 64-slice multidetector computed tomography (MDCT) was undertaken to provide optimal depiction of the PDA and main pulmonary artery (PA) and confirmed a widely patent PDA measuring 2.0 cm in largest diameter (Figure …A 28-year-old man presented to the emergency department with progressive shortness of breath complicated by large hemoptysis. At 3 months of age, he had been diagnosed with a “hole in his heart” in the Ukraine and had undergone 3 coronary catheterizations (2 in Russia, 1 in India; results unavailable). His parents had declined options for treatment. After stabilization in the emergency department, he was transferred to coronary care, where echocardiography demonstrated a persistent ductus arteriosus (PDA) measuring 0.9×2.0 cm with a right-to-left shunt consistent with Eisenmenger syndrome. Left ventricular function was severely impaired (ejection fraction=21%). Cardiac 64-slice multidetector computed tomography (MDCT) was undertaken to provide optimal depiction of the PDA and main pulmonary artery (PA) and confirmed a widely patent PDA measuring 2.0 cm in largest diameter (Figure …