Sheila J. Carroll

Age Less Than Two Years Is Not a Risk Factor for Mortality After Mitral Valve Replacement in Children

BACKGROUND Outcomes for mitral valve replacement (MVR) in the pediatric population are generally reported as poor, particularly in patients less than 2 years old. We compared long-term morbidity and mortality associated with MVR between patients less than 2 years old and patients 2 to 18 years old. METHODS We evaluated pediatric patients undergoing MVR from March 1990 to November 2007 at our institution. Morbidity and mortality was compared between patients less than 2 years and patients 2 to 18 years old. Primary endpoints measured were postoperative survival, long-term survival, reoperation, cerebrovascular accident or transient ischemic attack, and significant bleeding events. RESULTS Forty-five patients underwent 54 MVRs. Median age was 3.1 years; 18 patients were under 2 years. Median follow-up time was 5.4 years. There was no statistically significant difference between long-term or short-term survival between the two age groups, with 30-day survival of 89% (younger patients) versus 100% (older patients), and 10-year survival of 82% (younger patients) versus 85% (older patients). Freedom from reoperation for the younger age group was 40% at 10 years versus 96% for the older patients, p = 0.003. CONCLUSIONS In our population, there was no statistically significant difference in survival between patients less than 2 years and patients 2 to 18 years. In children undergoing MVR, age less than 2 years remains a risk factor for reoperation but not for mortality.

Repair of left ventricular diverticulum in setting of Cantrell's syndrome.

A 2 WEEK OLD GIRL, BORN AT 38 WEEKS GESTATION, had been noted on prenatal ultrasonic screening to have an omphalocele. Subsequent to birth, a superficial pulsatile mass was present, extending from the subxiphoid region into the omphalocele. Computed tomographic scanning with contrast (Fig. 1) showed a septated diverticulum (black arrow, white arrow indicates septum) which extended caudally from the left ventricle through the anterior defect in the diaphragm. Crosssectional echocardiography with colour Doppler in the four chamber view (Fig. 2) demonstrated a midline heart, patency of the oval fossa, and the pulsatile diverticulum (arrow, arrowhead indicates septum) extending from the left ventricle (LV) into the omphalocele. The septum within the diverticulum was separate from the true ventricular septum. The right ventricle (RV) was also seen in this view. On the 21st day of life, elective operative repair of the diverticulum was performed on cardiopulmonary bypass through a midline sternal incision. There was no abnormality in the sternum itself. The diverticulum (LVD) was exposed at its junction with the left ventricular apex (Fig. 3), resected, and closed with pledgeted sutures (Fig. 4, arrow) with the expectation that the devitalized tissue will eventually atrophy. The diaphragmatic and pericardial defects were also repaired. Postoperative recovery was unremarkable, and the patient was discharged five days after operation. This patient satisfied four of the five criterions for the syndrome first described by Cantrell in 1958, which is characterized by midline thoracoabdominal

Preoperative diagnosis of co-existing divided left atrium and tetralogy of Fallot

We present the eighth published case of divided left atrium co-existing with tetralogy of Fallot. This is the first report of preoperative echocardiographic diagnosis of this unusual combination of defects. Demonstration of a partition within the left atrium is imperative for successful repair of the combined lesions. We draw attention to the need for careful echocardiography in patients where an obstruction to pulmonary venous drainage would dramatically affect the outcome subsequent to surgical correction.

Hypoplastic left heart syndrome with restrictive atrial septum and advanced heart block documented with a novel fetal electrocardiographic monitor

Hypoplastic left ventricle with congenital heart block has been reported previously in a fetus with concurrent left atrial isomerism and levo‐transposition of the great arteries. We present the unusual case of an infant diagnosed in utero with hypoplastic left heart syndrome, a restrictive atrial septum and advanced heart block but with D‐looping of the ventricles and no atrial isomerism. In addition, fetal heart rhythm was documented with the assistance of a new fetal electrocardiographic monitor. Copyright

KCNQ1/KCNE1 Macromolecular Signaling Complex: Channel Microdomains and Human Disease

Autonomic nervous system control of heart rate and cardiac contractility, through sympathetic and parasympathetic activity, is a fundamental property of the cardiovascular system. Exercise or emotional stress stimulates the sympathetic nervous system (SNS), resulting in a rapid and dramatic increase in heart rate, which, in order to ensure adequate diastolic filling time, is accompanied by a concomitant reduction of the ventricular action potential duration (APD) and the corresponding QT interval on the electrocardiogram (EKG). Defective regulation of cardiac electrical activity in the face of SNS activity can lead to arrhythmias. 1