Timothy M. Cordes

Surgical management of complete atrioventricular septal defects: A twenty-year experience

Creation of a competent left atrioventricular valve is a cornerstone in surgical repair of complete atrioventricular septal defects. To identify risk factors for mortality and failure of left atrioventricular valve repair and to determine the impact of cleft closure on postoperative atrioventricular valve function, we retrospectively analyzed hospital records of 203 patients between January 1974 and January 1995. Overall early mortality was 7.9%. Operative mortality decreased significantly over the period of the study from 19% (4/21) before 1980 to 3% (2/67) after 1990 (p = 0.03). Ten-year survival including operative mortality was 91.3% +/- 0.004% (95% confidence limit): all survivors are in New York Heart Association class I or II. Preoperative atrioventricular valve regurgitation was assessed in 203 patients by angiography or echocardiography and was trivial or mild in 103 (52%), moderate in 82 (41%), and severe in 18 (8%). Left atrioventricular valve cleft was closed in 93% (189/203) but left alone when valve leaflet tissue was inadequate and closure of the cleft might cause significant stenosis. Reoperation for severe postoperative left atrioventricular valve regurgitation was necessary in eight patients, five of whom initially did not have closure of the cleft and three of whom had cleft closure. Six patients had reoperation with annuloplasty and two patients required left atrioventricular valve replacement. Five patients survived reoperation and are currently in New York Heart Association class I or II. On most recent evaluation assessed by angiography or echocardiography (a mean of 59 months after repair), left atrioventricular valve regurgitation was trivial or mild in 137 of the 146 survivors (94%) examined; none had moderate or severe left atrioventricular valve stenosis. By multiple logistic regression analysis, strong risk factors for early death and need for reoperation included postoperative pulmonary hypertensive crisis, immediate postoperative severe left atrioventricular valve regurgitation, and double-orifice left atrioventricular valve. These results indicate that complete atrioventricular septal defects can be repaired with low mortality and good intermediate to long-term results. Routine approximation of the cleft is safe and has a low incidence of reoperation for left atrioventricular valve regurgitation.

Surgical management of hypoplastic left heart syndrome

BACKGROUND The treatment of infants with hypoplastic left heart syndrome has been challenging and controversial. METHODS To assess the operative management and intermediate-term outcome, we retrospectively analyzed our surgical experience with 50 newborns with hypoplastic left heart syndrome operated on between January 1989 and June 1995. RESULTS Surgical palliation with a first-stage Norwood operation was offered to 28 patients. The remaining 22 infants were initially listed for heart transplantation, and 15 underwent the operation. Ten of the 15 recipients are alive, and all are in New York Heart Association class I. Seven infants underwent a Norwood procedure after being on the list for transplantation for 12 to 42 days. A total of 34 patients underwent Norwood procedures with one operation aborted because of inoperable anatomy. Two infants who survived the first-stage Norwood operation underwent subsequent heart transplantation and are currently doing well. The 1-year mortality rate for heart transplantation was 18% (3/17) versus 50% (17/34) for the Norwood procedure. Risk factors for early mortality after a Norwood procedure include longer circulatory arrest time (> 50 minutes), preoperative acidosis (pH < 7.20), larger systemic-pulmonary artery shunt (> or = 4 mm), diminutive ascending aorta (< or = 2.0 mm), and anatomic subtype of aortic and mitral atresia. The 1-year survival rate for the Norwood procedure improved from 36% for the patients operated on during 1989 through 1992 to 75% during 1993 to mid-1995 (p = 0.005). Of the 17 survivors of a first-stage Norwood operation, 10 have undergone the second stage (bidirectional Glenn procedure), and 7 have completed a Fontan procedure. Heart transplantation results have also improved, with no deaths since 1992. CONCLUSIONS Both the Norwood procedure and heart transplantation have encouraging early to intermediate results in infants with hypoplastic left heart syndrome. Hypoplastic left heart syndrome should be managed selectively on the basis of cardiac morphology, donor availability, and family wishes. Development of a flexible program involving the use of both procedures may aid in the successful management of infants with hypoplastic left heart syndrome.

ACCF/AHA/AAP recommendations for training in pediatric cardiology

SUMMARY It is vital to the future intellectual health of cardiovascularmedicine and the welfare of pediatric patients with cardiovas-cular disease that all future pediatric cardiologists be familiarwith the principles and tools of research. Training in researchrequires the intense involvement of productive and establishedinvestigators. Those trainees preparing for a career in investi-gative cardiology require a carefully developed but flexibleeducational plan that will permit them to be successful in theirresearch careers over an extended period. REFERENCES 1. Sonnenblick EH, Ryan TI, Starke RD. Task force 7: training incardiovascular research. J Am Coll Cardiol 1995;25:25–8.2. Roberts R, Alexander RW, Loscalzo J, Williams RS. Task force 7:training in cardiovascular research. Available at: http://www.acc.org/clinical/training/cocats2.pdf 2002. Accessed August 10, 2004.3. NHLBI Task Force Report on Pediatric Cardiovascular Diseases.Available at: http://www.nhlbi.nih.gov/resources/docs/pediatric_cvd.pdf.Accessed August 10, 2004.

Second-Line Treatment of Fetal Supraventricular Tachycardia Using Flecainide Acetate

Digoxin has been an effective treatment for fetal supraventricular tachycardia (SVT), but second-line therapy remains more controversial. Thirty-seven cases of fetal SVT were identified that received digoxin as first-line therapy. Seventeen fetuses (46%) converted to and maintained normal sinus rhythm. Flecainide was used in 13/15 patients requiring second-line therapy; 12/13 (92%) converted to sinus rhythm. Of seven hydropic fetuses, five required second-line therapy and were then successfully converted with flecainide. The improved efficacy of flecainide was statistically significant with a p value <0.01. Complete follow-up was available in 13 digoxin-treated and in 12 second-line therapy infants. Prolonged or multiple drug therapy for postnatal arrhythmia management was required in 3/13 (23%) patients in the digoxin group and in 8/12 (67%) patients requiring second-line therapy. This demonstrated a correlation between the need for second-line fetal therapy and more complex postnatal management with a p value of 0.003. Digoxin remains an effective first-line therapy in the treatment of fetal SVT. Flecainide is an effective second-line therapy, especially in the face of fetal hydrops. Use of second-line therapy in fetal SVT is a predictor of complex postnatal course, and these patients should be followed more closely.

Late onset of pulmonary hypertension after successful Mustard surgery for d-transposition of the great arteries.

Late-onset pulmonary hypertension is a serious complication of Mustard repair for d-transposition of the great arteries. This debilitating complication occurs in 7% of patients who survive to adulthood, even in the face of normal or near-normal postoperative pulmonary pressure.

Right Atrial Primary Cardiac Osteosarcoma

AbstractA previously healthy 14-year-old boy with an atypically located primary cardiac osteosarcoma filling the right atrium with significant extension into both systemic and pulmonary venous structures presented with a clinical picture of heart failure. Imaging showed a large right atrial mass with posterior extension into right pulmonary veins and inferior extension into the inferior vena cava causing near total obstruction of systemic venous return to the heart. Bone marrow biopsy showed no evidence of marrow involvement by metastatic malignancy. Histopathology was a high-grade osteosarcoma. Partial tumor debulking was achieved via right atriotomy.

ACCF/AHA/AAP recommendations for training in pediatric cardiology: A report of the American College of Cardiology Foundation/American Heart Association/American College of Physicians Task Force on Clinical Competence (ACC/AHA/AAP Writing Committee to Develop Training Recommendations for Pediatric Cardiology)

SUMMARY It is vital to the future intellectual health of cardiovascularmedicine and the welfare of pediatric patients with cardiovas-cular disease that all future pediatric cardiologists be familiarwith the principles and tools of research. Training in researchrequires the intense involvement of productive and establishedinvestigators. Those trainees preparing for a career in investi-gative cardiology require a carefully developed but flexibleeducational plan that will permit them to be successful in theirresearch careers over an extended period. REFERENCES 1. Sonnenblick EH, Ryan TI, Starke RD. Task force 7: training incardiovascular research. J Am Coll Cardiol 1995;25:25–8.2. Roberts R, Alexander RW, Loscalzo J, Williams RS. Task force 7:training in cardiovascular research. Available at: http://www.acc.org/clinical/training/cocats2.pdf 2002. Accessed August 10, 2004.3. NHLBI Task Force Report on Pediatric Cardiovascular Diseases.Available at: http://www.nhlbi.nih.gov/resources/docs/pediatric_cvd.pdf.Accessed August 10, 2004.

Attempts to change culture in a line-obsessed pediatric world: The Riley Heart Center line initiative

Interventions to reduce central line-associated bloodstream infections in pediatric patients have not yet been validated. Unique approaches to reducing infection rates may be warranted in this population.