Robert K. Darragh

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.

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.

Cardiac transplantation in infants and children

Cardiac transplantation has become a more frequently used therapeutic modality for select cardiac pathology in infants and children. Since June 1986, 30 pediatric patients (19 male and 11 female) ranging in age from 4 days to 15 years (11 < or = 1 month old) have undergone orthotopic cardiac transplantation at our institution. Indications included idiopathic cardiomyopathy (n = 8), hypoplastic left heart syndrome (n = 13), and other forms of complex congenital heart disease (n = 9). There have been four operative and three late deaths only in the groups with hypoplastic left heart syndrome and other forms of complex congenital heart disease. Cumulative survival is 77% after a mean follow-up of 30 months (range, 6 to 77 months). Three operative deaths were attributable to pulmonary hypertension, and the other was due to pulmonary hemorrhage. Two late deaths were secondary to allograft rejection, and the third was due to infection. There has been uniform survival in the group with idiopathic cardiomyopathy, and intermediate-term survival rates are 78% and 62% in the groups with complex congenital heart diseases and hypoplastic left heart syndrome, respectively (p = 0.15). Although longer-term results are necessary, orthotopic cardiac transplantation appears to be an acceptable mode of therapy for endstage heart disease in the pediatric age group and technically can be performed despite complex malformations of the great arteries or atria.

Increase of B‐type Natriuretic Peptide from Baseline Increases the Risk of Death or Retransplant in Pediatric Cardiac Transplant Patients, Midterm Results

UNLABELLED BACKGROUND; Several studies have demonstrated the utility of B-type natriuretic peptide (BNP) in the months following cardiac transplant. The purpose of this study was to analyze longitudinal BNP data in pediatric cardiac transplant patients and determine the efficacy of BNP in routine follow-up of transplant to help predict the adverse event of death or re-transplant. METHODS From October 2002 to July 2007, 53 pediatric cardiac transplant patients were treated in an unmatched case-control study. Along with routine studies, BNP values were obtained at regular intervals. Six patients were excluded due to recent transplant, and three patients were excluded due to poor compliance. A baseline BNP was established for each subject utilizing the mean of all BNP values during year 2 post-transplant, or the first year of measured BNP in those patients whose transplant preceded the study by more than 1 year (time from transplant to first BNP 7.0 +/- 3.5 years). The median BNP of all data points since transplant was utilized as an additional surrogate baseline. Univariate logistic regression was used to evaluate BNP versus other covariates on cardiac death. RESULTS Forty-four patients provided 1254 BNP data points spanning 173 patient years. Nine (20.5%) had an adverse event. Patients who experienced an adverse event had a higher baseline BNP (mean 365 +/- 290; median 375 +/- 352)) when compared to patients without an event (mean 128 +/- 78; median 121 +/- 62; p = 0.04 and p = 0.06)). All subjects with adverse events had a BNP value >or= 250 during the 90 days preceding the event compared to 32.4% of those who did not (odds ratio: 23.13, p < 0.01). A log fold increase in the BNP value compared to the baseline median BNP is a risk for cardiac death in the subsequent 90-day period (OR: 6.82, 95% confidence interval: 1.25-37.11, p = 0.03). CONCLUSION Routine BNP monitoring in the post-cardiac transplant pediatric patient allows for the determination of a median BNP, which can be used as a baseline. A log fold increase from the median BNP, or a BNP value >or= 250, increases the risk of death or re-transplant and suggests a 90-day period of heightened clinical surveillance, perhaps necessitating increased medication or re-listing for repeat transplant.

Quality of life following paediatric heart transplant: are age and activity level factors?

BACKGROUND We evaluated whether quality of life correlates to age and activity in children following heart transplantation. In addition, quality of life in children following heart transplantation was compared with previously reported values in children with congenital heart disease. Quality of life remains an important aspect of therapy. METHODS The Pediatric Quality of Life Inventory Generic Core Scales and Cardiac Module were administered to 14 children who had previously undergone heart transplantation. Patients wore a pedometer for 7 days to assess daily activity. RESULTS The age at assessment was 13.1±1.9 years. The patients were 7.1±5.7 years post heart transplantation. There was a negative correlation between age at first heart transplantation and emotional (r=-0.64; p<0.05) and school function (r=-0.57; p<0.05). A negative correlation between patients age at assessment and perceived physical appearance existed (r=-0.53; p<0.05). Daily steps negatively correlated with cognitive (r=-0.58; p<0.05), physical (r=-0.63; p<0.05), emotional (r=-0.62; p<0.05), and school function (r=-0.66; p<0.01). Heart transplantation patients reported better scores for treatment and symptoms (p<0.05) but lower physical health scores (p<0.01) than those with moderate congenital heart disease. CONCLUSIONS Paediatric heart transplantation patients reported overall similar quality of life as patients with moderate congenital heart disease. Children receiving heart transplants at an older age may require additional emotional and educational support. Heart transplantation patients with higher activity levels may be more aware of their physical, emotional, and cognitive limitations, and thus score lower on these quality of life indicators.

Severe airway obstruction from a bronchial cast after cardiac transplantation

Plastic bronchitis is a rare and difficult to treat disease process in patients with congenital heart disease. Cardiac transplantation has been used increasingly to reverse this process, especially in single ventricle physiology. This case report demonstrates a foreseeable complication after cardiac transplantation in such a patient.

Management of Complications Caused by a Massive Left Ventricle Tumor in a Neonate

We report the case of a neonate born with a giant fibroma occupying the entirety of her left ventricle. Owing to the extensive resection, her postoperative course was complicated by severely diminished left ventricular function and complete heart block necessitating extracorporeal support. Ultimately, cardiac resynchronization therapy was used, after which the infants ventricular function gradually improved and she was successfully discharged to home.