Current and emerging pharmacotherapies for the treatment of pulmonary arterial hypertension in infants.

Introduction: Pulmonary hypertension (PH) is a complex condition that encompasses an array of underlying disease processes and affects a diverse population of infants, including those with congenital heart disease, congenital diaphragmatic hernia, persistent PH of the newborn, and those with lung disease such as bronchopulmonary dysplasia. While there are treatments available to adults with PH, limited data exists for infants, especially for the newer medications. Therapies that target the three main pathophysiologic pathways of pulmonary hypertension appear to benefit infants, but which are best for each individual disease process is unclear.

Characteristics and Outcomes of Patients Discharged Directly Home From the Pediatric Intensive Care Unit.

Patients admitted to the pediatric intensive care unit (PICU) typically transfer to an acute care floor prior to discharge (ACD). Various circumstances, including rapid clinical improvement, technology dependence, or capacity constraints, may lead to discharge directly to home from a PICU (DDH). This practice has been studied in adult intensive care units, but research is lacking for PICU patients.

Increasing Seizures, Encephalopathy, and Hypothermia in an 18-Year-Old Woman.

An 18-year-old woman with a history of focal epilepsy presents with increasingly frequent seizures, encephalopathy, multiple laboratory abnormalities, and hypothermia. During her hospital course, her symptoms waxed and waned. Multiple etiologies of her symptoms were considered, but the spontaneous resolution of her symptoms and an abnormal MRI of the brain revealed the final diagnosis.

Beyond the Syndrome: Extensive Congenital Abnormalities in an Infant With Trisomy 21.

Herein we discuss the clinical course and subsequent autopsy of a female infant with trisomy 21 with balanced Rastelli Type "C" complete atrioventricular septal defect (AVSD), tetralogy of Fallot and right aortic arch with mirror image branching pattern who underwent a palliative right modified Blalock-Taussig-Thomas shunt (mBTTS) for hypoxemia from progressive right ventricular outflow tract obstruction. The baby was found to have multiple concomitant pathologic findings not typically seen with this constellation of cardiac anatomy. Autopsy revealed significant abdominal adhesions with near-complete stenosis of the transverse colon.

Left main coronary artery atresia in an infant with Shone's complex.

A 6-month-old infant with Shone's complex was found to have left main coronary artery atresia during evaluation for recurrent subaortic stenosis with depressed left ventricular function. The ventricular function improved after surgical subaortic resection without coronary re-vascularisation. This case demonstrates first the rare finding of left main coronary artery atresia and second that coronary re-vascularisation is not necessarily required in all cases of left main coronary artery atresia.

Dissociation of hemodynamic and electrocardiographic indexes of myocardial ischemia in pigs with hibernating myocardium and sudden cardiac death.

Many survivors of sudden cardiac death (SCD) have normal global ventricular function and severe coronary artery disease but no evidence of symptomatic ischemia or infarction before the development of lethal ventricular arrhythmias, and the trigger for ventricular tachycardia (VT)/ventricular fibrillation (VF) remains unclear. We sought to identify the role of spontaneous ischemia and temporal hemodynamic factors preceding SCD using continuous telemetry of left ventricular (LV) pressure and the ECG for periods up to 5 mo in swine (n = 37) with hibernating myocardium who experience spontaneous VT/VF in the absence of heart failure or infarction. Hemodynamics and ST deviation at the time of VT/VF were compared with survivors with hibernating myocardium as well as sham controls.

Brief sympathetic activation precedes the development of ventricular tachycardia and ventricular fibrillation in hibernating myocardium.

Background: Hibernating myocardium develops inhomogeneity in myocardial sympathetic innervation with spontaneous sudden cardiac death (SCD) because of ventricular fibrillation (VF). The triggers and prodromal arrhythmias initiating SCD in this substrate are unknown.

Methods: Swine chronically instrumented with a proximal left anterior descending coronary artery stenosis underwent placement of an implantable telemetry unit capable of continuously recording digitized electrocardiogram and left ventricular pressure signals at 1 kHz in conscious unrestrained animals for periods of up to 5 months.