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The preparticipation screening for athlete participation in sports typically entails a comprehensive medical and family history and a complete physical examination. A 12-lead electrocardiogram (ECG) can increase the likelihood of detecting cardiac diagnoses such as hypertrophic cardiomyopathy, but this diagnostic test as part of the screening process has engendered considerable controversy. The pro position is supported by argument that international screening protocols support its use, positive diagnosis has multiple benefits, history and physical examination are inadequate, primary prevention is essential, and the cost effectiveness is justified. Although the aforementioned myriad of justifications for routine ECG screening of young athletes can be persuasive, several valid contentions oppose supporting such a policy, namely, that the sudden death incidence is very (too) low, the ECG screening will be too costly, the false-positive rate is too high, resources will be allocated away from other diseases, and manpower is insufficient for its execution. Clinicians, including pediatric cardiologists, have an understandable proclivity for avoiding this prodigious national endeavor. The controversy, however, should not be focused on whether an inexpensive, noninvasive test such as an ECG should be mandated but should instead be directed at just how these tests for young athletes can be performed in the clinical imbroglio of these disease states (with variable genetic penetrance and phenotypic expression) with concomitant fiscal accountability and logistical expediency in this era of economic restraint. This monumental endeavor in any city or region requires two crucial elements well known to business scholars: implementation and execution. The eventual solution for the screening ECG dilemma requires a truly innovative and systematic approach that will liberate us from inadequate conventional solutions. Artificial intelligence, specifically the process termed "machine learning" and "neural networking," involves complex algorithms that allow computers to improve the decision-making process based on repeated input of empirical data (e.g., databases and ECGs). These elements all can be improved with a national database, evidence-based medicine, and in the near future, innovation that entails a Kurzweilian artificial intelligence infrastructure with machine learning and neural networking that will construct the ultimate clinical decision-making algorithm.

An 11-year-old boy with serologically confirmed Chlamydophila pneumoniae infection presented with clinical, laboratory, and echocardiographic changes consistent with myopericarditis. No reports on C. pneumoniae myopericarditis in children are found in the medical literature. The boy, previously healthy, presented with fever, rash, constitutional symptoms, elevated acute phase reactants, elevated cardiac enzymes, and high brain natriuretic peptide levels. Hemodynamic instabilities, including hypotension and mild hypoxia, were noted. Two-dimensional echocardiographic findings showed mildly depressed left ventricular systolic function and small pericardial effusion. Requiring inotropic support, the boy was treated with azithromycin 10 mg/kg once daily for 7 days and a single dose of intravenous immunoglobulin 2 g/kg. He recovered fully with improved left ventricular systolic function before hospital discharge. An early definitive diagnosis is essential to knowing the etiology of pediatric myocarditis. Specific therapy may play role in the management and prognosis of this disorder.

We describe a female infant with complex single ventricle physiology who had undergone median sternotomy for placement of a right-sided systemic-to-pulmonary artery shunt, division of a patent ductus arteriosus, and left pulmonary artery augmentation. Her early postoperative course was complicated by cardiac arrest requiring institution of extracorporeal membrane oxygenation (ECMO) support. The brain natriuretic peptide (BNP) levels acutely improved after left ventricular decompression by insertion of a left atrial cannula. In this setting BNP levels may be an indicator of left ventricular stretch and are potentially a useful index to monitor left ventricular distension.

Severe mitral regurgitation predicts poor outcomes in adults with left ventricular dysfunction. Frequently, adult patients now undergo initial mitral valve surgery instead of heart transplant. Pediatric data are limited. This study evaluates the efficacy of mitral valve surgery for severe mitral regurgitation in children with dilated cardiomyopathy. This is a single-institution experience in seven children (range, 0.5-10.9 years) with severe mitral regurgitation and dilated cardiomyopathy who underwent mitral valve surgery between January 1988 and February 2005, with follow-up to January 2006. Children with dilated cardiomyopathy had a depressed fractional shortening preoperatively (24.4% +/- 6.1%) that remained depressed (22.9% +/- 7.6%) 1.3 +/- 1.2 years after surgery (p = 0.50). Left ventricular end-diastolic (6.5 +/- 1.5 to 4.8 +/- 1.8 z-scores, p < 0.01) and end-systolic (6.8 +/- 1.5 to 5.5 +/- 2.1 z-scores, p < 0.05) dimensions improved. Hospitalization frequency had a median decrease of 6.0 hospitalizations per year (p < 0.02). Three patients were transplanted 0.2, 2.4, and 3.5 years after surgery. There was no perioperative mortality. Mitral valve surgery in children with dilated cardiomyopathy was performed safely and improved symptoms, stabilizing ventricular dysfunction in most patients. Mitral valve surgery should be considered prior to heart transplant in children with dilated cardiomyopathy and severe mitral regurgitation.

Congenital aortic stenosis is a relatively common cardiac anomaly encountered in approximately 5% of all children with heart disease. The Ross procedure is increasingly used for replacement of the aortic valve in children. We report a 12-year-old boy who was born with congenital aortic stenosis secondary to a bicommissural aortic valve. The patient underwent open valvotomy in infancy and aortic valvuloplasty 2 years later. Residual/recurrent stenosis prompted referral for aortic valve replacement, and he underwent an autologous Ross procedure, in which the aortic root was replaced with a pulmonary autograft and the repaired aortic valve was used to restore right ventricular-to-pulmonary artery continuity. The postoperative course was unremarkable. Nitroprusside, esmolol, and labetolol were used to control postoperative hypertension. He was discharged 4 days after surgery on oral furosemide and aspirin, and he has had no cardiovascular symptoms during follow-up. Recent echocardiography demonstrated mild right ventricular outflow tract obstruction with a peak velocity of 3.6 m/sec, with a gradient of 42 mmHg and moderate pulmonary insufficiency. There was no left ventricular outlet tract obstruction or aortic insufficiency.

The addition of new agents to the armamentarium of treatment options for heart failure in pediatric patients is exciting and challenging. Administration of these therapies to pediatric patients will require careful scrutiny of the data and skilled application. Developmental changes in drug metabolism, excretion, and distribution are concerning in pediatric patients, and inappropriate evaluation of these parameters can have disastrous results. Manipulation of the neurohormonal pathways in heart failure has been the target of most recently developed pharmacologic agents. Angiotensin receptor blockers (ARBs), aldosterone antagonists, beta-blockers, and natriuretic peptides are seeing increased use in pediatrics. In particular, calcium sensitizing agents represent a new frontier in the treatment of acute decompensated heart failure and may replace traditional inotropic therapies. Endothelin receptor antagonists have shown benefit in the treatment of pulmonary hypertension, but their use in heart failure is still debatable. Vasopressin antagonists, tumor necrosis factor inhibitors, and neutral endopeptidase inhibitors are also targeting aspects of the neurohormonal cascade that are currently not completely understood. The future of pharmacologic therapies will include pharmacogenomic studies on new and preexisting therapies for pediatric heart failure. The education and skill of the practitioner when applying these agents in pediatric heart failure is of utmost importance.

This study sought to determine the potential of recombinant B-type natriuretic peptide (nesiritide) for the treatment of pediatric decompensated heart failure. Nesiritide is a widely used and effective treatment for decompensated heart failure (HF) in adults, but its safety and efficacy in pediatric patients is unclear. Outcomes of 55 separate nesiritide infusions of varying durations in 32 patients (13 males and 19 females; mean age, 8.01 years; range, 0.01-20.4) were evaluated prospectively. All patients received nesiritide in the intensive care unit. The starting dose (0.01 microg/kg/min) was titrated to a maximum of 0.03 microg/kg/min. All patients were monitored for clinical signs and symptoms, hemodynamics, urine output, electrolytes, oxygen requirements, and oral intake. Functional status was assessed by patients and/or their parents. All patients successfully underwent initiation and titration of nesiritide infusion. No hypotension or arrhythmias were noted during 478 cumulative days of therapy. Nesiritide was given safely with vasoactive medications. Mean urine output improved from 2.35 +/- 1.71 cc/kg/hr on the day before nesiritide initiation (baseline) to 3.10 +/- 1.94 cc/kg/hr on day 4 of treatment (p < 0.01). Serum creatinine decreased from 1.04 to 0.92 mg/dl (p = 0.096), mean central venous pressure from 13 to 7 mmHg (p = 0.018), and mean weight from 30.4 to 29.7 kg (p < 0.001) with therapy. Thirst, as subjectively assessed by patients old enough to respond, decreased with infusion in 31 of 42 cases (74%). Mean New York Heart Association functional class improved significantly (p < 0.001). Nesiritide infusion, alone or in combination, is a safe treatment for decompensated HF in pediatric patients. It is associated with decreased thirst and improved urine output and functional status, and it may be efficacious in the treatment of pediatric HF.

We report a neonate with vein of Galen malformation (VGM) who developed congestive heart failure (CHF) early after birth. Serial changes in plasma B-type natriuretic peptide (BNP) following an endovascular embolization procedure for VGM were mirrored in his clinical CHF status. The plasma BNP level markedly increased to 1800 pg/ml (normal, <100 pg/ml) in accordance with the severity of CHF. It rapidly decreased to 356 pg/ml during the first week after endovascular embolization for VGM. In the following 3 weeks there was an unexpected upward trend in plasma BNP despite echocardiography revealing normal biventricular function. After additional evaluation and treatment for CHF, BNP decreased again and the patient's clinical status concurrently improved. The patient was discharged with a normal BNP level. Monitoring serial plasma BNP provides valuable information regarding the need for additional evaluation or treatment of newborns with CHF and may be used to document improvement.

We report a case of an infant with complex congenital heart disease who was placed on captopril for afterload reduction following cardiac surgery and subsequently developed pulmonary infiltrates with eosinophilia. The patient was readmitted with symptoms of rhinorrhea, poor feeding, and decreased activity level. She was found to have diffuse pulmonary infiltrates on chest radiograph and a marked peripheral eosinophilia without leukocytosis. After discontinuing captopril and starting systemic steroids, her symptomatology rapidly improved, and her eosinophilia and radiographic abnormalities both resolved.

In recent years, left ventricular noncompaction (LVNC) has been recognized as a distinct form of cardiomyopathy with its own clinical presentation and natural history. More than 100 cases of LVNC have been described in children. Although LVNC has been described in association with metabolic disorders such as Fabry's disease or genetic disorders such as Roifman's syndrome, this case represents the first report of LVNC in a child with trisomy 13.

This is the case report of a 13-year-old male who developed vasopressor-resistant hypotension after cardiac surgery for endocarditis. As norepinephrine resulted in aggravation of the preexisting ventricular arrhythmia, vasopressin was used to maintain blood pressure. The vasopressin continuous infusion was started at 0.00002 units/kg/min and titrated up to 0.0003 U/kg/min. This low dose led to resolution of hypotension without causing side effects. As the appropriate indication and dose of vasopressin is not established, the cautious use of vasopressin in children is recommended.

The following is a case report of a 1-month-old patient who developed adverse hemodynamic sequelae during the use of nitric oxide (NO) in the postoperative period for pulmonary hypertension after correction of total anomalous pulmonary venous return. At the time of diagnosis, the patient had evidence of systemic right ventricular pressures estimated by continuous-wave Doppler. He was sedated and paralyzed for hyperventilation in preparation for surgery and underwent pulmonary vein confluence to left atrial anastomosis. Postoperative pulmonary hypertension was managed by hyperventilation, sedation, and paralysis until a sudden onset of systemic-level pulmonary pressure required NO therapy. Satisfactory results were obtained in minutes, but a rebound pulmonary hypertension occurred with concomitant systemic hypertension and no radiographic changes. We suspected left atrial hypertension secondary to a sudden increase in pulmonary blood flow to an noncompliant left ventricle. Discontinuation of NO resulted in stabilization of the hemodynamic profile of the patient and he continued to be managed with paralysis, hyperventilation, and sedation. Based on this experience we suggest that NO should be used with caution in patients with obstructive lesions at the atrial level prior to surgery (mitral valve stenosis and cor triatriatum) or in patients with a poorly compliant left ventricle (cardiomyopathy and left ventricular dysfunction). These entities are unable to tolerate a sudden increase in pulmonary blood return thus creating paradoxical pulmonary hypertension.