Lee B. Beerman

Update on Cardiovascular Implantable Electronic Device Infections and Their Management A Scientific Statement From the American Heart Association

Despite improvements in cardiovascular implantable electronic device (CIED) design, application of timely infection control practices, and administration of antibiotic prophylaxis at the time of device placement, CIED infections continue to occur and can be life-threatening. This has prompted the study of all aspects of CIED infections. Recognizing the recent advances in our understanding of the epidemiology, risk factors, microbiology, management, and prevention of CIED infections, the American Heart Association commissioned this scientific statement to educate clinicians about CIED infections, provide explicit recommendations for the care of patients with suspected or established CIED infections, and highlight areas of needed research.

Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography A Scientific Statement From the American Heart Association

Background— Acute rheumatic fever remains a serious healthcare concern for the majority of the world’s population despite its decline in incidence in Europe and North America. The goal of this statement was to review the historic Jones criteria used to diagnose acute rheumatic fever in the context of the current epidemiology of the disease and to update those criteria to also take into account recent evidence supporting the use of Doppler echocardiography in the diagnosis of carditis as a major manifestation of acute rheumatic fever. Methods and Results— To achieve this goal, the American Heart Association’s Council on Cardiovascular Disease in the Young and its Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee organized a writing group to comprehensively review and evaluate the impact of population-specific differences in acute rheumatic fever presentation and changes in presentation that can result from the now worldwide availability of nonsteroidal anti-inflammatory drugs. In addition, a methodological assessment of the numerous published studies that support the use of Doppler echocardiography as a means to diagnose cardiac involvement in acute rheumatic fever, even when overt clinical findings are not apparent, was undertaken to determine the evidence basis for defining subclinical carditis and including it as a major criterion of the Jones criteria. This effort has resulted in the first substantial revision to the Jones criteria by the American Heart Association since 1992 and the first application of the Classification of Recommendations and Levels of Evidence categories developed by the American College of Cardiology/American Heart Association to the Jones criteria. Conclusions— This revision of the Jones criteria now brings them into closer alignment with other international guidelines for the diagnosis of acute rheumatic fever by defining high-risk populations, recognizing variability in clinical presentation in these high-risk populations, and including Doppler echocardiography as a tool to diagnose cardiac involvement.

Blade atrial septostomy: collaborative study.

During the past 4 years, five institutions have collaborated in evaluating the efficacy of blade atrial septostomy. The procedure was performed in 52 patients, including 31 with transposition of the great arteries, 10 with mitral atresia, five with tricuspid atresia and six with miscellaneous anomalies. The patients ages ranged from 1 day to 12 years (mean 13 months). Improvement occurred in 41 of 52 patients (79%). Four patients had an intact interatrial septum, and blade atrial septostomy was successfully performed by a transseptal technique. One patient died from a lacerated left atrial wall; other complications occurred in four patients. Blade atrial septostomy is an effective palliative procedure, even when the interatrial septum is thickened or intact.

Extracorporeal membrane oxygenation for postcardiotomy cardiogenic shock in children

Size limitations and technical barriers prohibit the use of many conventional mechanical circulatory support systems for postcardiotomy ventricular dysfunction in pediatric populations. Extracorporeal membrane oxygenation (ECMO), frequently used to treat neonatal respiratory failure, can provide cardiac support and is effective treatment of postoperative myocardial failure in children. From 1981 to 1987, 10 patients aged 2 days to 5 years were maintained on ECMO for 15 to 144 hours (mean duration, 92 +/- 16 hours) after cardiotomy. Operative procedures included repair of tetralogy of Fallot (2 patients), closure of a ventricular septal defect (2), the Senning procedure for transposition of the great arteries (1 patient), repair of interrupted aortic arch with closure of a ventricular septal defect (1), repair of a partial atrioventricular septal defect (2), closure of a ventricular septal defect with excision of an anomalous muscle bundle (1), and the Fontan procedure (1). Venoarterial ECMO was established in all 10 children. Six patients underwent transthoracic right atrium-ascending aorta cannulation, 3 had right internal jugular vein-right common carotid artery cannulation through a cervical incision, and 1 had right internal jugular vein-left axillary artery cannulation. Eight of the 10 patients were successfully weaned from ECMO, and 7 are long-term survivors. There were 3 deaths; 1 was caused by cardiac and acute renal failure complicated by sepsis two days after decannulation, another occurred 19 days after atrioventricular septal defect repair, and 1 was caused by massive pulmonary hemorrhage. Major hemorrhage developed in 3 patients while on ECMO; 2 required premature decannulation for mediastinal bleeding from operative sites and ultimately survived, and 1 died of respiratory failure as a result of endobronchial bleeding. We conclude that the use of ECMO in pediatric populations for transient postoperative ventricular dysfunction improves survival with limited overall morbidity.

Coronary arteriosclerosis in pediatric heart transplant survivors: Limitation of long-term survival

Because coronary atherosclerosis after heart transplantation has been a limiting problem in long-term survival of adults, we reviewed the coronary angiograms, and autopsy data when available, from 21 of 30 children who underwent orthotopic heart transplantation and survived the perioperative period. Six patients had coronary atherosclerosis, and five of these patients died 6 months to 3 years after heart transplantation. The late deaths were sudden and unexpected. Coronary angiography demonstrated several types of lesions, including concentric narrowing, tubular segmental lesions, and abrupt obliteration of major coronary vessels. Risk factors assessed included hypertension, hyperlipidemia, cytomegalovirus infection, type of immunosuppressive regimen, number of rejection episodes, and major histocompatibility antigen mismatches. Only the frequency and duration of rejection episodes seemed to be more prevalent in the patients in whom coronary atherosclerosis developed. Despite the benefits of heart transplantation in treating children with end-stage heart disease, coronary atherosclerosis may limit long-term survival. We suggest that these children should undergo serial coronary angiography to identify those at risk for subsequent events related to coronary artery disease.

Tetralogy of Fallot with absent pulmonic valve: analysis of 17 patients.

Seventeen patients with tetralogy of Fallot (TF) and absent pulmonic valve were seen at Childrens Hospital of Pittsburgh from 1958 to 1981. In 7 patients, severe respiratory symptoms developed due to bronchial compression by an aneurysmal pulmonary artery, and 6 died. In 4 patients, moderate respiratory symptoms developed. Six patients remained free of respiratory distress. Medical and surgical management of patients with respiratory distress in infancy has been frustrating and frequently unsuccessful. A 4-month-old infant with respiratory distress underwent complete intracardiac repair with valve insertion and has subsequently survived with relief of symptoms. Considering the high mortality rate in this group of patients, further attempts at aggressive surgical repair in infancy for patients with severe respiratory symptoms is warranted. An anatomic review of pathologic specimens with this anomaly confirmed previous reports of the frequent association of absence of the ductus arteriosus in patients with tetralogy of Fallot and absent pulmonary valve. The exception is noted in patients with discontinuity between the right and left pulmonary arteries in which a ductus arteriosus may be present supplying the isolated left pulmonary artery.

Rest and exercise right and left ventricular function late after the mustard operation: Assessment by radionuclide ventriculography☆

To evaluate ventricular function late after atrial repair of transposition of the great arteries (TGA), 26 asymptomatic patients had rest and exercise radionuclide ventriculography performed a mean of 9 years (range 5 to 15) after undergoing the Mustard operation. The mean resting right (systemic) ventricular (RV) ejection fraction (EF) was 0.50 +/- 0.10 (+/- 1 standard deviation); the RVEF was less than 0.45 in 8 patients. With exercise the RVEF increased in 9 patients and either failed to increase or decreased in 15 (including all 8 patients with resting values less than 0.45). The weight-adjusted work load performed was a first predictor of RV exercise response (sensitivity 87%, specificity 92%); patients whose RVEF increased did more work. The mean resting left (pulmonary) ventricular (LV) EF was 0.58 +/- 0.09; the LVEF was less than 0.50 in 3 patients. With exercise the LVEF increased in 14 patients and did not increase in 10 (including all 3 with resting values less than 0.50). The presence of complex ventricular arrhythmia documented on Holter monitoring was a first predictor of failure of the LVEF to increase with exercise (sensitivity 84%, specificity 71%). The patients age, operative age, postoperative interval, residual arterial desaturation, preoperative large ventricular septal defect or pulmonary stenosis, postoperative pulmonary stenosis or superior vena caval obstruction, or performance of a second open-heart procedure was predictive of the rest or exercise EF of either ventricle.

Infective Endocarditis in Childhood: 2015 Update A Scientific Statement From the American Heart Association

In 2002, the American Heart Association (AHA) published “Unique Features of Infective Endocarditis in Childhood,”1 which reviewed epidemiology, pathogenesis, diagnosis, clinical and laboratory findings, treatment, and prevention of infective endocarditis (IE) with particular attention to children. Since that time, other AHA reports have focused on new recommendations for treatment of IE in adults (in 20052) and on major changes regarding prevention of IE (in 20073). This document updates these issues and other concerns regarding IE, with specific attention to the disease as it affects infants and children. In particular, the impact of increased survival for children with congenital heart disease (CHD) on the epidemiology of IE is updated, and newer tools useful for diagnosis and treatment in the pediatric population are reviewed. This review emphasizes changing management perspectives and discussion of new agents that have utility for treatment of resistant organisms. In addition, proper use of the diagnostic microbiology laboratory remains critical to the diagnosis and management of children with IE, and newer diagnostic guidelines that have improved sensitivity and specificity for confirming the diagnosis of IE will be reviewed. Because of improved infrastructure available for home intravenous therapy, an update on outpatient management, an increasingly accepted approach for stable patients who are at low risk for complications, will also be discussed. Finally, since the publication of the last AHA document on pediatric IE, recommendations for prevention of IE have been modified substantially, and the most current recommendations are incorporated from the perspective of pediatric cardiovascular concerns. In pediatrics, there are not likely to be any randomized controlled trials for treatment of IE, which posed a challenge for the writing group in compiling recommendations. Therefore, many of the indications are based on consensus. In cases of strong consensus that an intervention be considered as standard-of-care practice with …

Hemodynamic function after the mustard operation for transposition of the great arteries

Pre- and postoperative cardiac catheterization data and cinenangiocardiograms of 82 patients who survived the Mustard operation for transposition of the great arteries (TGA) were reviewed. The post-operative catheterizations were performed 20 days to 10 years after operation (mean 2.5 years). Forty-six patients (56%) had no or insignificant associated cardiac lesions, whereas 36 (44%) had ventricular septal defect, pulmonary stenosis, or both, and required surgical intervention at the time of the Mustard operation. Postoperatively, 11 patients (13%) had significant systemic venous obstruction. Of the 11 patients, 6 required reoperation, and 2 patients had evidence of restenosis or complete obstruction in the superior vena cava after reoperation. In most patients, superior vena caval obstruction was well tolerated even in the presence of high pressure in the superior vena cava. Pulmonary venous obstruction occurred in 5 patients (6%), 3 of whom had no clinical symptoms despite severe pulmonary venous obstruction, although all had radiographic evidence of pulmonary venous congestion. The incidence of obstruction was drastically reduced after the Mustard operation was modified to include routine enlargement of the pulmonary venous atrium. Tricuspid regurgitation was uncommon (10%), but did occur in patients who had transatrial closure of a ventricular septal defect. Preoperatively, left ventricular outflow obstruction occurred in 38%. In 12 patients an attempt was made to relieve the obstruction at surgery. The 6 patients who had localized obstruction had a good result, but patients with more diffuse narrowing of left ventricular outflow had little or no relief of obstruction. Mild to moderate left ventricular outflow gradients regressed spontaneously in most patients after the Mustard operation.

Echocardiographic findings before and after liver transplantation

Echocardiographic studies were performed in 73 patients with various types of chronic liver disease. They were 0.5 to 19 years old (mean 5). Thirteen patients underwent follow-up echocardiography 1 to 13 months (mean 6) after liver transplantation. Preoperatively 60 patients (82%) showed evidence of high cardiac output (cardiac index greater than 4 liters/min/m2); these patients manifested increased left ventricular (LV) and left atrial dimensions and a thickened LV posterior wall. Transvenous contrast echocardiographic study confirmed the presence of intrapulmonary arteriovenous shunting in 4 patients. Studies after liver transplantation revealed a reduced LV end-diastolic dimension in 12 patients. Cardiac index was reduced a mean of 35% after transplantation (p less than 0.001). This study suggests that liver transplantation improves common hemodynamic abnormalities in chronic liver disease.