Michael D. Freed

Guidelines for the Management of Patients With Valvular Heart Disease Executive Summary A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients With Valvular Heart Disease)

This executive summary and recommendations appears in the November 3, 1998, issue of Circulation . The guidelines in their entirety, including the ACC/AHA Class I, II, and III recommendations, are published in the November 1, 1998, issue of the Journal of the American College of Cardiology . Reprints of both the full text and the executive summary and recommendations are available from both organizations. During the past 2 decades, major advances have occurred in diagnostic techniques, the understanding of natural history, and interventional cardiological and surgical procedures for patients with valvular heart disease. The information base from which to make clinical management decisions has greatly expanded in recent years, yet in many situations, management issues remain controversial or uncertain. Unlike many other forms of cardiovascular disease, there is a scarcity of large-scale multicenter trials addressing the diagnosis and treatment of valvular disease from which to derive definitive conclusions, and the literature represents primarily the experiences reported by single institutions in relatively small numbers of patients. The Committee on Management of Patients With Valvular Disease was given the task of reviewing and compiling this information base and making recommendations for diagnostic testing, treatment, and physical activity. These guidelines follow the format established in previous American College of Cardiology/American Heart Association (ACC/AHA) guidelines for classifying indications for diagnostic and therapeutic procedures: Class I: Conditions for which there is evidence and/or general agreement that a given procedure or treatment is useful and effective Class II: Conditions for which there is conflicting evidence and/or a divergence of opinion about the usefulness/efficacy of a procedure or treatment IIa. Weight of evidence/opinion is in favor of usefulness/efficacy IIb. Usefulness/efficacy is less well established by evidence/opinion. Class III: Conditions for which there is evidence and/or general agreement that the procedure/treatment is not useful and in some cases …

Diagnosis and therapy of Kawasaki disease in children.

Adnan S. Dajani, MD, Chairman; Kathryn A. Taubert, PhD; Michael A. Gerber, MD; Stanford T. Shulman, MD; Patricia Ferrieri, MD; Michael Freed, MD; Masato Takahashi, MD; Fredrick Z. Bierman, MD; Adolf W. Karchmer, MD; Walter Wilson, MD; Shahbudin H. Rahimtoola, MD; David T. Durack, MD, DPhil, (Liaison, Infectious Diseases Society of America); Georges Peter, MD (Liaison, American Academy of Pediatrics, Committee on Infectious Diseases), Members, Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association

ACC/AHA 2008 Guideline Update on Valvular Heart Disease: Focused Update on Infective Endocarditis A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines: Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons

Robert O. Bonow, MD, MACC, FAHA, Chair Blase A. Carabello, MD, FACC, FAHA Kanu Chatterjee, MB, FACC Antonio C. de Leon, Jr, MD, FACC, FAHA David P. Faxon, MD, FACC, FAHA Michael D. Freed, MD, FACC, FAHA William H. Gaasch, MD, FACC, FAHA Bruce W. Lytle, MD, FACC, FAHA Rick A. Nishimura, MD,

Guidelines for long-term management of patients with Kawasaki disease. Report from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.

Long-term management of patients with Kawasaki disease should be tailored to the degree of coronary arterial involvement. This committee has made recommendations for each risk level about antiplatelet and anticoagulant therapy, physical activity, follow-up assessment by a pediatric cardiologist or primary care physician, and the appropriate diagnostic procedures that may be performed to evaluate cardiac disease. The risk level for a given patient with coronary arterial involvement may change over time because of changes in coronary artery morphology. The recommendations for management presented here are intended as practical interim guidelines until additional prospective or retrospective data are compiled to define more clearly the natural history of Kawasaki disease.

Side effects of therapy with prostaglandin E1 in infants with critical congenital heart disease.

The case reports of 492 infants with critical congenital *-rdiac disease treated with prostaglandin E1 (PGE1) were reviewed to determine the nature and incidence of intercurrent medical events. Forty-three percent of the infants had at least one such event, but only half of these were related to PGE1 and the majority required only minor changes in management. Cardiovascular events were the most common (18% incidence), with cutaneous vasodilation and edema occurring more frequently during intraaortic infusion than during i.v. infusion. Central nervous system events were reported in 16% of the patients. Respiratory depression was reported in 12%, and was particularly common in infants weighing less than 2.0 kg at birth (42%). Hematologic, infectious and renal events appeared for the most part to be unrelated to PGE1. The overall mortality (excluding 19 patients with hypoplastic left-heart syndrome) was 31%; the mortality for the patients with critical coarctation or interruption of the aortic arch was nearly twice that for the cyanotic infants (50% vs 27%). No death was attributed to PGE1 administration.During infusion of PGE1, arterial blood pressure and respiratory activity should be monitored carefully and appropriate supportive steps taken if hypotension or respiratory depression occurs. The development of fever or jitteriness may require reduction of the infusion rate and, in view of the possible increased incidence of infections, the prophylactic use of antibiotics is recommended.

Prostaglandin E1 infants with ductus arteriosus-dependent congenital heart disease.

Between January 1976 and June 1979, 492 infants with ductus arteriosus-dependent congenital heart disease (385 cyanotic and 107 acyanotic) received prostaglandin E, (PGE,) in 56 centers in the United States as part of a protocol sponsored by the Upjohn Company. In the infants with cyanotic congenital heart disease, the mean Pao2 for the group increased from 26.7 mm Hg before to 38.5 mm Hg during infusion (p < 0.001). Infants older than age 4 days had a higher preinfusion Pao2 and a smaller increase in Pao2. Infants weighing more than 4 kg at birth and alkalotic infants (pH > 7.45) had a smaller increase in Pao2. No differences in response were found with respect to mode of administration (intra-arterial or i.v.), gender, maternal age, or preinfusion Paco2. PGE, provides excellent palliation for infants with ductus arteriosus- dependent cyanotic congenital heart disease.Of the 107 infants with acyanotic congenital heart disease, 46 had juxtaductal coarctation and 34 had interruption of the aortic arch. Clinical improvement occurred in about 80% in each group. In the infants with aortic interruption, descending aortic blood pressures increased and the pressure differences across the ductus arteriosus decreased markedly. In the infants with coarctation, descending aortic blood pressures increased and ascending aortic pressure decreased. The systolic pressure difference across the coarctation decreased markedly, from 45 to 9 mm Hg. Infants in whom the ductus arteriosus was closed before the infusion showed no beneficial effects. In infants in whom a constricted ductus arteriosus relaxed, the effect occurred more slowly than in infants with cyanotic heart disease. PGE, also provides excellent palliation in infants with aortic obstruction in which lower body perfusion is dependent on the ductus arteriosus.

Use of Hancock porcine xenografts in children and adolescents

Between January 1,1974 and December 31,1978,47 xenografts (8 aortic, 28 mitral, 4 tricuspid, 3 left atrioventricular valve in I-transposition of the great arteries and 3 aortic-mitral) were implanted in 44 patients, aged 10/12 to 20 5/12 years (median 12 2/12). All eight patients with aortic valve replacement survived and were followed up for 22.5 ± 10.4 months (mean ± standard error of the mean). At postoperative cardiac catheterization in three patients, the peak systolic ejection gradient ranged between 24 and 38 mm Hg and the valve area index between 0.71 and 1.15 cm2/m2. Among 26 patients who underwent mitral valve replacement, 5 died operatively and 2 died 2 months postoperatively, leaving 19 long-term survivors who were followed up for 26.2 ± 15.6 months. In the 11 patients who underwent postoperative cardiac catheterization, the mean diastolic gradient was 6.1 ± 2.7 mm Hg and the mean valve index 1.79 ± 0.76 cm2/m2. Severe subaortic obstruction, due to the prosthetic mitral valve, occurred in three of five patients less than 5 years old. Among four patients who underwent tricuspid valve replacement there were no operative deaths; complete heart block occurred in three. At postoperative catheterization in two patients, the mean diastolic gradient was 3 and 5 mm Hg, and the valve index 1.32 and 1.7 cm2/m2, respectively. Three patients with I-transposition of the great arteries who underwent replacement of the systemic atrioventricular valve were followed up for 12 to 37 months. At postoperative catheterization the mean diastolic gradient was 0 to 4 mm Hg and the valve index ranged from 1.68 to 1.88 cm2/m2. Both aortic and mitral valves were replaced in three children. One died at operation and one 6 months after operation. The survivor has complete heart block but is in New York Heart Association functional class I. Eight xenografts (6 mitral, 1 left atrioventricular valve in I-transposition of the great arteries and 1 aortic) failed, requiring replacement 22 to 68 months after implantation. All were calcified to varying degrees. Valve failure began presenting 22 months after implantation, and of 25 valves implanted for 20 months or longer, 8 have been replaced. These 8 patients were younger (10.3 ± 0.2 years) at initial valve replacement than the other 17 patients (15.0 ± 0.3 years) followed up for 20 months or longer (p <0.05). High levels of γ-carboxyglutamic acid (GLA), a calciumbinding amino acid, were found in all six calcified xenografts analyzed, whereas none was found in a fresh porcine aortic valve or in an unimplanted xenograft. GLA-containing protein, laid down after implantation, may play a role in xenograft calcification.

The Use of Heparinization to Prevent Arterial Thrombosis after Percutaneous Cardiac Catheterization In Children

Thrombosis at the site of arterial puncture is a common and serious complication of percutaneous cardiac catheterization in children. A double-blind study was therefore designed to test the efficacy of heparin administered during catheterization in reducing this complication. One hundred and sixty-one unselected children over one year of age were studied. Prior to catheterization, the pulse amplitudes were measured in both legs using a Pulse Volume Recorder (a standardized oscillometer). Immediately after arterial cannulation, heparin, 1 mg/kg, or a placebo was administered. On the morning following catheterization, the pulse amplitude in both legs was remeasured and a pulse volume index calculated using the uncatheterized leg as a control. Patients in whom the catheterized leg was cold, with poor capillary filling and absent arterial pulses four hours after the completion of catheterization, were started on intravenous infusion of heparin. If no improvement occurred within 48 hours, embolectomy was performed.There was no evidence of arterial compromise to the catheterized extremity to any patient older than 10 years. Of 77 children ten years of age or younger, systemic heparinization postcatheterization was required for a pulseless extremity in 40% (15/37) of those in the placebo group and 8% (3/40) in the heparin group (P = 0.003). Only 5% (2/40) of children in the heparinized group had a pulse volume index of less than 50%, compared to 27% (10/37) in the placebo group (P = 0.003). Embolectomy was performed in seven of the 37 children in the placebo group and none of 40 in the heparin group (P = 0.01). No complications related to the use of heparin were found. We conclude that heparin administered during percutaneous catheterization is effective in preventing arterial thrombosis in children ten years of age or younger and should be routinely administered unless a specific contraindication to its use exists.

Exercise-Induced Hypertension After Surgical Repair of Coarctation of the Aorta

To study exercise-induced hypertension after surgical repair of coarctation of the aorta, 30 patients aged 6 to 30 years (median 14) were exercised to exhaustion on a treadmill using the Bruce protocol. Twenty patients with hemodynamically insignificant heart disease served as controls. Systolic blood pressure was measured simultaneously in the arm and leg before and immediately after exercise. There was no significant difference between the control and coarctectomy groups with regard to age at exercise, duration of exercise, maximal heart rate or serum lactate after exercise. Blood pressure in the arm increased significantly after exercise in both groups (control group: 110 [range 90 to 140] to 135 [range 114 to 164] mm Hg, P P P P P r = 0.91, P This high correlation suggests that the residual obstruction across the site of the coarctectomy causes the observed postexercise systolic hypertension in the arm. Exercise testing with blood pressure measurements should be performed in all children after repair of coarctation of the aorta. In those whose postexercise systolic pressure in the arm exceeds 200 mm Hg, catheterization should be considered to measure central aortic pressure with exercise because reoperation, antihypertensive medication or restriction of severe physical effort may be necessary.

Repair of Tetralogy of Fallot in Infancy

Our present treatment philosophy for congenital cardiac defects is aimed, whenever possible, at early repair before the heart, lungs, and brain become irreversibly affected and while they retain their developmental potential. Consequently, primary repair of tetralogy of Fallot (TOF) is preferred to a two-stage approach. In neonates and infants with hypoxic spells and/or persistent hypoxemia, repair is advised irrespective of age or weight. Because of the gratifying experience with repair of TOF in this age group, we have also steadily decreased the age of elective repair primarily to avoid the secondary hypertrophic changes within the right ventricle. We now recommend elective repair during the first 2 years of life. Surgery is postponed to 4 or 5 years of age in those patients who have anatomic RV-PA discontinuity. This is a review of our surgical experience with symptomatic infants with TOF.